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Central Annals of Otolaryngology and Rhinology
Cite this article: Oliveira EB, de Barros Baptista MAF, de Liz
AA, dos Passos Martins DAN (2015) Cochlear Implantation in a
Patient with Kearns-Sayre Syndrome: Case Report and Literature
Review. Ann Otolaryngol Rhinol 2(11): 1070.
*Corresponding authorMarco Antônio Ferraz de Barros Baptista,
Departament of Otolaryngologist, University of São Paulo, Rua
Sílvio Marchione, nº 3-20, Bairro: Vila Universitária, CEP:
17.012-900 Bauru - SP, Brazil, Email:
Submitted: 15 October 2015
Accepted: 03 November 2015
Published: 05 November 2015
ISSN: 2379-948X
Copyright© 2015 de Barros Baptista et al.
OPEN ACCESS
Keywords•Cochlear implantation•Twin•Kearns-Sayre
syndrome•Genetic deafness•Ensorineural hearing loss•Mitochondrial
disorders
Case Report
Cochlear Implantation in a Patient with Kearns-Sayre Syndrome:
Case Report and Literature ReviewEduardo Boaventura Oliveira, Marco
Antônio Ferraz de Barros Baptista*, Alcebíades Alves de Liz and
Danilo Augusto Nery dos Passos MartinsDepartment of Otolaryngology,
University of São Paulo, Brazil
Abstract
Introduction: The Kearns-Sayre syndrome (KSS) belongs to the
group of mitochondrial diseases. Cells that require more energy
intake, such as muscle, nerve, retinal and cochlear, are most
commonly affected.
The established diagnostic criteria are: 1) the age of onset
before 20 years old (100%), 2) progressive external ophthalmoplegia
(100%), 3) pigmentary retinopathy (100%), and 4) at least one of
the following: heart block, cerebellar ataxia (84%) and protein>
100 mg/dL in the cerebrospinal fluid. Additional features are the
presence of bilateral sensorineural hearing loss and myopathy.
Genetic testing of mitochondrial DNA can confirm the diagnosis.
The aspect of muscle biopsy is typical, showing ragged red fibers
(RRF).
Objectives: Present a literature review and report the cochlear
implantation in a patient with KSS.
Case Report: ACVF, female, was born in 1989 from a gemelar
pregnancy. At the age of ten years, the patient began manifesting
hypoacusia associated with a continuous and bilateral tinnitus. In
2000, the patient was evaluated by a medical geneticist because of
palpebral ptosis, progressive ophthalmoplegia, reduction in muscle
mass, facies myopathica, an alteration in the index-nose test and
slight restriction of ocular motricity. A muscular biopsy revealed
RRF. The genetic test performed showed deletion of mitochondrial
DNA compatible with a diagnosis of KSS. Her twin sister also had
the diagnosis of the syndrome based on the same tests.
The patient showed a deterioration of hypoacusia even after the
adaptation of the hearing aid. During the tone audiometric exam, it
was seen that the patient had evolved towards a bilateral and
profound deafness. The results of the complete audiological
evaluation enabled the realization of Cochlear Implantation.
INTRODUCTIONThe Kearns-Sayre syndrome (KSS) belongs to the group
of
mitochondrial diseases, which have as their main feature the
tendency to affect the tissues that depend substantially on the
oxidative phosphorylation process. Cells that require more energy
intake, such as muscle, nerve, retinal and cochlear, are most
commonly affected [1].
The mitochondrion is a cytoplasmic organelle. It is known that,
during the process of fertilization, only the ovum contributes
to the cytoplasm of the embryo and, therefore, the mitochondrial
DNA (mtDNA) descends exclusively from the maternal lineage.
Mutations in mtDNA can be inherited or occur sporadically; affect
both sexes and are not transmitted to offspring compulsorily, since
the random replication of this material originates from a few
copies, known as the effect of “genetic bottleneck”. The variable
phenotypic expression is explained by a threshold effect, which
heteroplasmy with a proportion of at least 80-90% of mutant mtDNA
tissue, as a general rule, determine organ dysfunction and clinical
symptoms [2-4].
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Congenital hearing loss can be classified in terms of etymology,
contemplating environmental and genetic causes, accounting for 50%
each. Within the genetic causes, syndromic forms are implicated in
about 30% of cases, in contrast to 70% of forms of nonsyndromic
deafness. The genetic forms of inheritance are dominant autosomal
(77%), recessive autosomal (21%), X-linked (~1%) and mitochondrial
(~ 1%) [5]. Despite the fact that the mitochondrial form is rare,
sensorineural hearing loss (SNHL) is found in 42% to 70% of
patients with mitochondrial disorders [6,7] and 97% in KSS [4]. A
study of Japanese group with SNHL identified a prevalence of 3%
mtDNA mutation [8].
Mitochondrial deafness obeys the symmetry law of hearing loss of
Langenbeck, exhibits a sensorineural pattern, and also affects
characteristically high frequencies. The progressive deterioration
can occur at rates of 1.5 - 7, 9dB a year and usually evolves
towards profound SNHL [9,10]. The occurrence in individuals with
mutations of an early reduction of oxidative phosphorylation due to
aging and tenuous mtDNA repair mechanisms is postulated. The
decrease in ATP levels causes dysfunction in endocochlear potential
provided by Na + K + ATP pumps of stria vascularis. In addition,
the fact that the outer hair cells receive indirect metabolic
support of Deiters cells, particularly those that are most
metabolically active and located in the basal coil (tonotopy high
frequency), would provide enhanced susceptibility to damage
[3,11].
The KSS is presented as a multisystemic, degenerative and rare
disease with an estimated incidence of 1-3 per 100.000 in the
population. De novo mutation is more prevalent than that of
maternal inheritance, occurring either in the mother’s oocyte or
very early in embryonic development. The mtDNA deletion in large
scale from 1.1 to 10 kilobases the mechanism associated in 90% of
cases. More than 150 different mtDNA deletions have been associated
with KSS. A deletion of 4977 bp known as m.8470_13446 del 4977 is
encountered most frequently. Large-scale duplications of mtDNA
coexist with deletions in some individuals with KSS [12].
The established diagnostic criteria are: 1) the age of onset
before 20 years old (100%), 2) progressive external ophthalmoplegia
(100%), 3) pigmentary retinopathy (100%), and 4) at least one of
the following: heart block, cerebellar ataxia (84%) and protein>
100 mg/dL in the cerebrospinal fluid. Additional features are the
presence of bilateral SNHL (97%), myopathy (94%), intellectual
deficit (86%), diabetes mellitus (13%), dysphagia,
hypoparathyroidism and renal tubular acidosis [12,13].
The clinical diagnosis must be confirmed by molecular studies,
both long-range PCR as southern blot, in leukocytes or muscle. Both
are recommended to perform from muscle biopsy sample because the
mutation may be undetectable in blood cells due to the possibility
of different level of heteroplasmy. The appearance of muscle biopsy
is typical, showing RRF with the modified Gomori trichrome stain.
Histochemical analysis demonstrates a hyperreactivity of fibers
with the succinate dehydrogenase reaction; there is also the
possibility of failure to stain with the histochemical reaction for
cytochrome c oxidase (COX) and decreased activity of respiratory
chain mtDNA [12].
OBJECTIVESPresent a literature review and report the
cochlear
implantation in a patient with Kearns-Sayre syndrome.
CASE REPORTACVF, female, was born in 1989 from a term and
gemelar
pregnancy, with a vaginal birth without complications, and a
weight of 2950 grams. After the birth, she remained hospitalized
for three days and lost 200 grams during this period. In her
gestational history, she reported that her mother received prenatal
accompaniment, but did not get an ultrasound during the pregnancy.
In her medical history, she states that at the age of 18 months she
was admitted to a hospital due to gastroenterocolitis and
dehydration, as well as for a urinary tract infection. At the age
of two years she was diagnosed with mumps and chickenpox. She
presented a few episodes of tonsilitis that were treated with
antibiotics. Her neuro-pshychomotor development was normal, in
accordance with age.
There is no consanguinity between her parents and there also are
no cases of deafness in her family history. Her twin sister also
was diagnosed with KSS and at present receives
otorhinolaryngological accompaniment at a different medical center
because of moderate and bilateral SNHL.
In June of 2000, the patient was evaluated by a medical
geneticist because of a complaint of palpebral ptosis and
progressive ophthalmoplegia that commenced in 1993. According to
data from the consultation, she was diagnosed with astigmatism,
hypermetropy, and visual fatigue. During the physical exam, she
exhibited a reduction in muscle mass with preserved motor force,
facies myopathica, intention tremor, dysmetria in the index-nose
test and dysdiadochokinesia suggesting cerebellar ataxia, a slight
restriction of ocular motricity, and a normal fundus examination.
There was no report of other affected members of the family.
With regard to the geneticists’ report, the following exames of
both sisters are within normal range: FTA-Abs (IgG and IgM), VDRL,
FAN, VHS, reative protein c, glycemia, and complete blood count. In
audiological evaluation showed moderate SNHL, predominantly of high
frequencies. The patient had no response in the examination of
Otoacoustic Emissions (OAE) and the examination of the Auditory
Brainstem Response (ABR) bilaterally. During the audiological
evaluation, the hypothesis of mitochondriopathy was considered and
the patient was referred to a muscular biopsy, genetic tests,
ophthalmological and cardiological exam.
A muscular biopsy, which was performed at the Federal University
of São Paulo (UNIFESP) in 2000, revealed a distribution of 80% of
type I muscular fibers with preserved mosaic, the presence of
atophy of polygonal fibers, and in the internal architecture, a
typical finding of KSS: presence of RRF. Hypertrophy was not seen,
nor was nuclear centralization, inflammation, necrosis, inclusion
or alterations of muscle spindles.
The genetic test performed twelve years later (2012) showed
deletion of mitochondrial DNA compatible with a diagnosis of KSS.
We not had access to the results of genetic testing, so we
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cannot mention what type of deletion found on examination. Her
twin sister also was diagnosed with KSS based on the clinical
findings, muscle biopsy and genetic test. We cannot prove that the
patients are identical twins because they do not have a DNA test or
an ultrasound done during pregnancy.
The retinal angiogram that was performed in 2010 exhibited,
during initial and intermediary phases of contrast,
hyperfluorescence and hypofluorescence on the posterior side of
both eyes, which is highly suggestive of focal atrophy of the
pigmented epithelium of the retina and areas of pigment,
respectively. The leakage of contrast was not visualized.
The result of the echocardiogram, which was done in 2012,
revealed mixomatose degeneration of the mitral valve with prolapse
of the anterior leaflet into the interior of the left atrium with
minimal reflux detected by color Doppler.
In relation to hearing, at the age of ten years, the patient
began manifesting hypoacusia associated with a continuous and
bilateral tinnitus. In 2000, she was submitted to an audiological
evaluation that consisted of a tone audiometry, speech audiometry,
immittance audiometry (tympanometry and the study of acoustic
reflexes), as well as a differential audiological evaluation, by
means of an electroacoustic and electrophysiological exam, being
OAE and ABR, respectively. The exams indicated severe and bilateral
SNHL and a hearing aid was adapted within the same year. The
patient was maintained under our care until 2002, when she decided
to attain assistance at a different hearing center that was closer
to her place of residence. Figure 1A shows the last tonal
audiometry conducted before the last follow-up.
During twelve years of accompaniment at another hearing center,
the patient showed a deterioration of hypoacusia even
after the adaptation of the hearing aid, which was maintained in
continuous usage. Consequently, she was referred yet again to our
hospital in August of 2014, which is considered to be a reference
in audiology, as well as for cochlear implantation evaluation.
During the otorhinlaryngological exam, an integral tympanic
membrane was observed and in the audiological exam, the patient
reported to have noticed better hearing in her right ear. During
the tone audiometric exam, it was seen that the patient had evolved
towards a bilateral and profound deafness (Figure 1B), according to
the established WHO criteria (WHO, 1997). Because of the degree of
hearing loss, it was not possible to execute the speech recognition
tests (SRT), such as Percentage Index of Speech Recognition (PISR).
It was then necessary to evaluate the Voice Detection Threshold
(VDT). During the immittance audiometry exam, a type A curve in the
right ear was seen, and the type Ad curve was seen in the left ear,
according to the Jerger classification scale [14]. There was an
absence of contralateral acoustic reflexes, as well as ipsilateral
reflexes in both ears. The examination of transient OAE and
distortion product OAE was absence of bilateral responses. ABR by
click stimulation demonstrated an absence of neural potential
bilaterally in 95dBNA and the presence of cochlear microphonism in
90 dBNA in both ears. The results of the complete audiological
evaluation enabled the realization of Cochlear Implantation.
In February of 2015, the patient was submitted to cochlear
implant surgery in her left ear. During the procedure, the tympanic
membrane was visualized and was integral and shiny. A
retroauricular incision of 3 cm was performed, followed by the
drilling of the mastoid bone, which permitted the visualization of
an integral and articulated bony chain. The facial nerve was
integral in its tympanic and mastoid trajectory. A posterior
tympanotomy was performed, maintaining as a reference the
Figure 1 A. Tonal audiogram performed in 2002 that exhibited
severe SNHL in the left ear and profound in the right ear. B. Tonal
audiogram conducted in February of 2015, pre-operational, that
demonstrated profound and bilateral SNHL. Legend: O: airway of the
right ear / : bone conduction of the left ear (in blue) / no
response to the given stimulus: ↙ right ear (in red) / ↘left ear
(in blue)
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short branch of the incus, facial nerve, and the corda tympani
nerve. Following that, a cochleostomy was done with a 1 mm drill
perpendicularly to the stapes tendon and it was seen that the
cochlea was in a normal state. After that, the total insertion of
electrodes was conducted (Figure 2) and an intra-operational
neurotelemetry was registered. The model used for the cochlear
implant in this surgery was HiRes90K™ Advantage cochlear implant,
by Advanced Bionics.
One month after the procedure, new exams were conducted, such as
telemetry, telemetry of neural responses, the study of the
stapedial reflex, electrode mapping, electrode activation, as well
as phonoaudiological evaluation. During a consultation after four
months of surgery, the patient mentioned great improvement of
quality of life with the cochlear implant. She is still using the
hearing aid in her right ear. During the tone audiometry exam, she
presented a hearing improvement of 55 dB during frequencies of
500Hz, 75 dB during 1000 Hz, and 95 dB during 2000 Hz and 4000 Hz
(Figure 3).
DISCUSSIONKearns-Sayre syndrome (KSS) was first described by
Thomas
P. Kearns and George Pomeroy Sayre in 1958 [15]. It is a rare
disease and to our knowledge, there are only three reported cases
of the disease published in the English language that were
submitted to cochlear implantation surgery. Furthermore, there is
only one report of twin brothers with the syndrome. There are no
reports in medical literature of female twins with the
syndrome.
The patient in our study presented the stipulated diagnostic
criteria established for KSS, because the initial symptoms due to
progressive ophthalmoplegia occurred before the age of twenty and
was associated with pigmentary retinopathy, as well as a cerebellar
ataxia. Also present were the bilaterally symmetric SNHL, a genetic
test showing a deletion in the mitochondrial
DNA, a muscular biopsy that exhibited a myopathic pattern, and
the presence of RRF, which is a typical finding of the disease.
Deletions of mtDNA, ranging in size from 1.1 to 10 kb are
associated with KSS, Pearson syndrome, progressive external
ophthalmoplegia (PEO), and rarely Leigh syndrome. In Pearson
syndrome, deletions are usually more abundant in blood than in
other tissues and PEO deletions are confined to skeletal muscle
[12].
Most mitochondrial diseases arise from a disruption in oxidative
phosphorylation, often due to a defect in one or more respiratory
complexes of the mitochondrial respiratory chain. A decreased
activity leading to a reduced cellular energy production in the
form of adenosine triphosphate (ATP), resulting in functional cell
impairment, oxidative cellular injury or even apoptosis.This
multienzyme system, located in the inner mitochondrial membrane, is
made up of five polypeptide complexes (I, II, III, IV, and V) and
two mobile electron carriers: coenzyme Q (quinone derivative with
ten isoprene units) and cytochrome c (a small, extrinsic protein).
Each complex consists of various subunits encoded by nuclear DNA,
which is imported into the mitochondria. The gene products, in a
process that is poorly understood, then combine to comprise the
inner mitochondrial respiratory chain [16,17].
Cytochrome c donates electrons from complex III to complex IV
(COX). Coenzyme Q10 (also known as ubiquinone) behaves as a
homogeneously pooled redox carrier between flavin dehydrogenases
and the cytochrome system, transferring reducing equivalents from
complexes I and II to complex III. It may also translocate protons
from the mitochondrial matrix to the intermembrane space,
contributing to the energy conservation occurring at coupling site
2 of the respiratory chain. Reduced coenzyme Q10 also acts as an
antioxidant, protecting
Figure 2 A radiography was immediately taken post-operationally,
with the white arrow pointing to the total insertion of electrodes
in the left cochlea.
Figure 3 Sound field audiometry performed with audiometer, model
MD622, four months after cochlear implant surgery showing great
improvement in hearing thresholds in the left ear.Legend: □ Hearing
threshold in the left ear.
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mitochondrial inner membrane lipids and proteins, and
mitochondrial DNA against oxidative damage. This coenzyme is
slightly decreased in KSS [16,17].
Lactic acidosis is caused by pyruvate accumulation when the
inner mitochondrial respiratory chain is dysfunctional. The
morphology of this occurrence is seen as RRF in muscle pathologic
specimens. As mentioned earlier, the mitochondria use genetic
products from mitochondrial DNA as well as from nuclear DNA.
Complex II, also labeled succinate dehydrogenase, is composed
exclusively of nuclear components. The ragged red appearance is a
classic morphologic feature of mitochondrial disease caused by high
levels of succinate dehydrogenase generated to compensate for low
levels of oxygen [16].
Abnormalities are observed with aging process: a loss of COX
activity and concomitant increase in succinate dehydrogenase
activity (succinate dehydrogenase hyperreactive regions, also known
as a ragged red phenotype) [18]. Consequently, the percentage of
RRF is variable in the syndrome.
Hearing impairment is a common feature of the KSS, however, can
be found less often in other mitochondriopathies as mitochondrial
encephalomyopathy with lactic acidosis and stroke-like episodes
(MELAS), PEO, mitochondrial myopathy (MM), maternally inherited
diabetes and deafness (MIDD), myoclonic epilepsy with ragged red
fibers (MERRF) [19]. There are other diseases that can cause
similar findings to those of KSS as ophthalmoplegia, weakness and
pigmentary retinopathy. The main differential diagnosis of KSS are
the Pearson Syndrome, MELAS, PEO and MERRF [20,21].
MELAS syndrome onset may occur early in infancy with a history
of developmental delay and learning disabilities. Patients may have
visual complaints due to ophthalmoplegia, blindness because of
optic atrophy and difficulties with night vision due to pigmentary
retinopathy. The absence of strokelike episodes (hallmark feature
of this disorder), episodes of seizures (tonic-clonic or myoclonic)
and visual abnormalities followed by hemiplegia decrease the
probability of this diagnosis for the patient of this report. Some
patients may experience hearing loss and diabetes [22].
In comparison with patients who have MELAS, patients with KSS
also present with RRF that are COX-negative because their
mitochondrial DNA genome is not synthesizing COX 2, an important
subunit of the mitochondrial genome. MELAS patients typically have
muscle biopsies revealing classic COX-positive ragged red fibers
[16]. Unfortunately, there was no information about the COX
activity in the muscle biopsy of our patient.
Pearson marrow-pancreas syndrome was first described in 1979 as
an often fatal disorder of infants with transfusion-dependent
sideroblastic anemia, vacuolization of hematopoietic precursors,
and exocrine pancreatic insufficiency [23]. It is now known to be a
rare, multisystemic, mitochondrial cytopathy with anemia,
neutropenia, and thrombocytopenia, as well as variable hepatic,
renal, and endocrine failure. Death usually occurs early in life,
often during metabolic crises marked by refractory severe lactic
acidosis during intercurrent, usually infectious illnesses [24].
Our patient had a complete blood count with normal
values and no history of pancreatic, hepatic, renal and
endocrine impairment.
PEO is a mitochondrial myopathy with drooping of the eyelids
(ptosis), paralysis of the extraocular muscles (ophthalmoplegia),
and variably severe proximal limb weakness. A few individuals with
PEO have other manifestations of KSS but do not fulfill all the
clinical criteria for the diagnosis. This situation is called “KSS
minus” or “PEO plus”. In PEO, mtDNA deletions are confined to
skeletal muscle. The disorder is relatively benign and compatible
with a normal life span.The multisystemic form is KSS; in the past,
KSS was also referred to as “ophthalmoplegia-plus,” a term now used
to describe individuals who have more than isolated myopathy but do
not fulfill the clinical criteria for KSS [12].
Our patient had a mitral valve prolapse displayed on the
Echocardiography exam. This finding is not common in mitochondrial
diseases. We found only one case report of a patient diagnosed with
mitochondrial disease and the presence of mitral valve prolapsed
[25]. The electrocardiogram did not show the presence of cardiac
blockage, a common finding in KSS.
There are few studies that show the possible alterations in the
auditory system of patients with mitochondrial diseases. Liu [19]
et al. (2014) evaluated the hearing of 73 patients with
mitochondrial diseases, and amongst them only two had KSS and both
patients had complaints of hearing loss. Of the 73 evaluated
individuals, 52 (71%) presented alterations of tone audiometry, and
in 51 of them the hearing deficit was symmetric. The only patient
that did not present symmetrical hearing loss had unilateral otitis
media at the moment of the evaluation, which generated an air-bone
gap. The patients mentioned in our case report presented symmetric
SNHL, corroborating with the Liu [19] et al. study in 2014.
With regard to the two patients with KSS that were mentioned in
the Liu [19] et al. study, one of them only had hearing loss in
high frequencies and the other presented moderate SNHL, with
symptoms worsening in high frequencies. The latter situation is
similar to the patient described in this report. One of the
patients with KSS mentioned in the study by Liu [19] et al.
presented a discrepancy between the findings in the pure tone
audiometry and ABR. During the audiometry, the average tone within
the range of 0.5, 1, 2 and 4 kHz (WHO standard) of 20 in the left
ear and 18 in the right ear was found, with a reduction during
acute frequencies. ABR demonstrated a disappearance in neural
response in the right ear and the presence of waves in the left ear
of 80 dB, with the value of increased absolute latency for I
wave.
These abnormalities suggested temporal non-synchronicity in
central auditory pathway or auditory cortex dysfunction, and they
should be diagnosed as auditory neuropathy spectrum disorder.
Recently, several case reports have shown that auditory neuropathy
can occasionally occur in some patients with mtDNA disease
[26,27].
Mitochondrial segregation during germline development follows a
bottleneck sampling effect, in which only a small fraction of the
mother’s mtDNA is shared with progeny. This limits the probability
that a mutation will be transferred to the next generation [16].
This can explain why risk of maternal
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transmission of mitochondrial DNA deletion disorder has been
estimated to be approximately 1 in 24 [28].
Interesting data reported by Khambatta [29] et al. (2014) is
that none of the 35 patients with KSS in their study presented a
familial history of the disease, which is a situation identical to
that of the twin sisters mentioned in our study. Mt DNA deletions
generally occur de novo in the mother’s oocyte or during
embryogenesis [12] and thus, usually cause disease in only one
family member. In addition to the first bottleneck sampling effect,
there is also a somatic bottleneck event. As the zygote progresses
to the multicellular stage (up to 128 cells) only approximately 3
of those cells will contribute to the formation of the fetus. The
other cells will be allocated to support, as extra-embryonic
tissue. This sampling effect can partially explain why different
patients have varying severities of disease [16].
The diversity of clinical presentations in KSS can be explained
by the fact that a single mtDNA mutation can result in the
expression of multiple phenotypes [29]. In compliance, Zeviani [30]
et al. (1988) conclued that multiple phenotypes (ranging from mild
to severe disease) were present with the same mtDNA mutation. The
mutated mtDNA coexists with normal molecules (heteroplasmy) and the
proportion of mutated to normal mtDNA correlates with the severity
of clinical symptoms [31]. Studies identified that mtDNA
heteroplasmy levels, mtDNA deletion size and location are all
important in understanding the expression and progression of
clinical disease [32]. This data also explains the fact that twins
with de same disease can have diverse variations in audiological
findings.
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Oliveira EB, de Barros Baptista MAF, de Liz AA, dos Passos
Martins DAN (2015) Cochlear Implantation in a Patient with
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Cite this article
http://www.ncbi.nlm.nih.gov/pubmed/25061332http://www.ncbi.nlm.nih.gov/pubmed/25061332http://www.ncbi.nlm.nih.gov/pubmed/25061332http://www.ncbi.nlm.nih.gov/pubmed/3412580http://www.ncbi.nlm.nih.gov/pubmed/3412580http://www.ncbi.nlm.nih.gov/pubmed/3412580http://www.ncbi.nlm.nih.gov/pubmed/17342029http://www.ncbi.nlm.nih.gov/pubmed/17342029http://www.ncbi.nlm.nih.gov/pubmed/17342029http://www.ncbi.nlm.nih.gov/pubmed/24277717http://www.ncbi.nlm.nih.gov/pubmed/24277717http://www.ncbi.nlm.nih.gov/pubmed/24277717
Cochlear Implantation in a Patient with Kearns-Sayre Syndrome:
Case Report and Literature ReviewAbstractIntroductionObjectivesCase
Report DiscussionReferencesFigure 1Figure 2Figure 3