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September 1996, Vol 122, No. 9 >
ARTICLE | September 1996

Audiovestibular Findings in Patients With Deafness Caused by a Mitochondrial Susceptibility Mutation and Precipitated by an Inherited Nuclear Mutation or Aminoglycosides FREE

Itzhak Braverman, MD; Lutfi Jaber, MD; Haya Levi, MA; Cahtia Adelman, MSc; Kathleen S. Arons, PhD; Nathan Fischel-Ghodsian, MD; Mordechai Shohat, MD; Josef Elidan, MD
Arch Otolaryngol Head Neck Surg. 1996;122(9):1001-1004. doi:10.1001/archotol.1996.01890210073016
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Objective:  To characterize the audiological and vestibular changes associated with a mitochondrial DNA mutation in an Arab-Israeli family and in other families with mitochondrial predisposition to aminoglycoside-induced hearing loss.

Design:  Evaluation of audiological (pure tone thresholds, speech reception thresholds, speech discrimination, tympanometry, acoustic reflex thresholds, tone decay, and auditory brain-stem evoked response recording) and vestibular (complete history, physical examination, and 2-channel electronystagmography) systems. In 5 patients, structural evaluation of the inner ear was done by magnetic resonance imaging.

Patients:  Fifteen members of an Arab-Israeli family, and 1 Chinese woman with the same mitochondrial DNA mutation who experienced hearing loss after short-term exposure to streptomycin.

Results:  Most of the patients had a profound hearing loss due to cochlear involvement. The hearing loss usually was not accompanied by notable peripheral vestibular dysfunction. In the patient with severe hearing loss after exposure to aminoglycoside, the vestibular function was completely normal.

Conclusions:  In most of the Arab-Israeli patients with congenital deafness, the vestibular system function was normal, in contrast to the frequency of vestibular abnormality among deaf children, which was described in the literature. This may be related to genetic predisposition to aminoglycoside-induced deafness.Arch Otolaryngol Head Neck Surg. 1996;122:1001-1004

REFERENCES

1 +
Attardi G, Schatz G.  Biogenesis of mitochondria . Annu Rev Cell Biol . 1988;;4: 289-333.
2 +
Wallace DC.  Diseases of the mitochondrial DNA . Annu Rev Biochem . 1992;; 61:1175-1212.
3 +
Giles RE, Blane H, Cann HM, Wallace DC.  Maternal inheritance of human mitochondrial DNA . Proc Natl Acad Sci U S A . 1980;;77:6715-6719.
4 +
Case JT, Wallace DC.  Maternal inheritance of mitochondrial DNA polymorphisms in cultured human fibroblasts . Somat Cell Mol Genet . 1981;;7:103-108.
5 +
Jaber L, Shohat M, Bu X, et al.  Sensorineural deafness inherited as a tissue-specific mitochondrial disorder . J Med Genet . 1992;;29:86-90.
6 +
Bu XD, Shohat M, Jaber L, Rotter JI.  A form of sensorineural deafness is determined by a mitochondrial and an autosomal locus: evidence from pedigree segragation analysis . Genet Epidemiol . 1993;;10:3-15.
7 +
Prezant TR, Agapian JV, Bohlman C, et al.  Mitochondrial ribosomal RNA mutation associated with both antibiotic-induced and nonsyndromic deafness . Nat Genet . 1993;;4:289-294.
8 +
Arving J.  Vestibular function in deafness and severe hardness of hearing . Acta Otolaryngol (Stockh) . 1955;;45:283-288.
9 +
Goldstein R, Landau WM, Kleffner FR.  Neurological assessment of some deaf and aphasic children . Ann Otolaryngol . 1958;;67:468-479.
10 +
Feinmesser M, Banberger-Thal L, Marcus P.  Cause of deafness in children . Harefuah . 1963;;64:294-297.
11 +
Sandberg LE, Terkildsen K.  Caloric tests in deaf children . Arch Otolaryngol . 1965;;81:350-354.
12 +
Argaman Z, Elidan J. The Vestibular Function in Children With Profound Sensorineural Hearing Loss. Jerusalem, Israel: Hebrew-University Hadassah Medical School; 1988. Thesis.
13 +
Higashi K.  Unique inheritance of streptomycin-induced deafness . Clin Genet . 1989;;35:433-436.
14 +
Hu DN, Qui WQ, Wu BT, et al.  Genetic aspect of antibiotic-induced deafness: mitochondrial inheritance . J Med Genet . 1991;;28:79-83.
15 +
Fischel-Ghodsian N, Prezant TR, Bu X, Oztas S.  Mitochondrial rhibosomal RNA gene mutation in patients with sporadic aminoglicoside ototoxicity . Am J Otolaryngol . 1993;;14:399-403.
16 +
Hutchin T, Haworth I, Higasgi K, Fischel-Ghodsian N, Stoneking M, Saha N, Amos K, Cortopassi G.  A molecular basis for human hypersensitivity to aminoglycoside antibiotics . Nucleic Acids Res . 1993;;21:4174-4179.
17 +
Bacino C, Prezant TR, Bu X, Fournier P, Fischel-Ghodsian N. Susceptibility mutations in the mitochondrial small rebosomal RNA gene in aminoglycoside induced deafness. Pharmacogenetics. In press.
18 +
Warchol ME, Lambert PR, Goldstein BJ, Forge A, Corwin JT.  Regenerative proliferation in inner ear sensory epithelia from adult guinea pigs and humans . Science . 1993;;259:1619-1621.

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1 +
Attardi G, Schatz G.  Biogenesis of mitochondria . Annu Rev Cell Biol . 1988;;4: 289-333.
2 +
Wallace DC.  Diseases of the mitochondrial DNA . Annu Rev Biochem . 1992;; 61:1175-1212.
3 +
Giles RE, Blane H, Cann HM, Wallace DC.  Maternal inheritance of human mitochondrial DNA . Proc Natl Acad Sci U S A . 1980;;77:6715-6719.
4 +
Case JT, Wallace DC.  Maternal inheritance of mitochondrial DNA polymorphisms in cultured human fibroblasts . Somat Cell Mol Genet . 1981;;7:103-108.
5 +
Jaber L, Shohat M, Bu X, et al.  Sensorineural deafness inherited as a tissue-specific mitochondrial disorder . J Med Genet . 1992;;29:86-90.
6 +
Bu XD, Shohat M, Jaber L, Rotter JI.  A form of sensorineural deafness is determined by a mitochondrial and an autosomal locus: evidence from pedigree segragation analysis . Genet Epidemiol . 1993;;10:3-15.
7 +
Prezant TR, Agapian JV, Bohlman C, et al.  Mitochondrial ribosomal RNA mutation associated with both antibiotic-induced and nonsyndromic deafness . Nat Genet . 1993;;4:289-294.
8 +
Arving J.  Vestibular function in deafness and severe hardness of hearing . Acta Otolaryngol (Stockh) . 1955;;45:283-288.
9 +
Goldstein R, Landau WM, Kleffner FR.  Neurological assessment of some deaf and aphasic children . Ann Otolaryngol . 1958;;67:468-479.
10 +
Feinmesser M, Banberger-Thal L, Marcus P.  Cause of deafness in children . Harefuah . 1963;;64:294-297.
11 +
Sandberg LE, Terkildsen K.  Caloric tests in deaf children . Arch Otolaryngol . 1965;;81:350-354.
12 +
Argaman Z, Elidan J. The Vestibular Function in Children With Profound Sensorineural Hearing Loss. Jerusalem, Israel: Hebrew-University Hadassah Medical School; 1988. Thesis.
13 +
Higashi K.  Unique inheritance of streptomycin-induced deafness . Clin Genet . 1989;;35:433-436.
14 +
Hu DN, Qui WQ, Wu BT, et al.  Genetic aspect of antibiotic-induced deafness: mitochondrial inheritance . J Med Genet . 1991;;28:79-83.
15 +
Fischel-Ghodsian N, Prezant TR, Bu X, Oztas S.  Mitochondrial rhibosomal RNA gene mutation in patients with sporadic aminoglicoside ototoxicity . Am J Otolaryngol . 1993;;14:399-403.
16 +
Hutchin T, Haworth I, Higasgi K, Fischel-Ghodsian N, Stoneking M, Saha N, Amos K, Cortopassi G.  A molecular basis for human hypersensitivity to aminoglycoside antibiotics . Nucleic Acids Res . 1993;;21:4174-4179.
17 +
Bacino C, Prezant TR, Bu X, Fournier P, Fischel-Ghodsian N. Susceptibility mutations in the mitochondrial small rebosomal RNA gene in aminoglycoside induced deafness. Pharmacogenetics. In press.
18 +
Warchol ME, Lambert PR, Goldstein BJ, Forge A, Corwin JT.  Regenerative proliferation in inner ear sensory epithelia from adult guinea pigs and humans . Science . 1993;;259:1619-1621.

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