Table 9.

Agents/Circumstances to Avoid: Select Genetic Disorders

GeneDisorderConcernCircumstance to Avoid
Jervel and Lange-Nielsen syndrome Prolonged QT intervalAnesthetic agents & medications that may further prolong the QT interval. This is particularly important for children undergoing cochlear implantation. Avoid activities that may precipitate syncopal episodes: competitive sports, amusement parks, jumping into cold water.
MT-RNR1 1Mitochondrial SNHL (See Nonsyndromic Hearing Loss and Deafness, Mitochondrial.)Sensitivity to aminoglycosides predisposes to hearing lossExposure (even intermittent) to aminoglycosides
MT-TS1 2
POU3F4 X-linked deafness (OMIM 304400)↑ risk that middle ear surgery can result in complete loss of hearingConductive hearing loss in this disorder is caused by stapedial fixation. Fenestration or removal of the stapes footplate at the time of middle ear surgery that results in an abnormal communication between the CSF & perilymph can lead to fluid leakage ("perilymphatic gusher") and complete loss of hearing.

CSF = cerebrospinal fluid; SNHL = sensorineural hearing loss


Virtually all persons w/m.1095T>C, m.1494C>T, or m.1555A>G pathogenic variants will have hearing loss after even intermittent exposure to aminoglycosides. Sensitivity to aminoglycosides related to several other variants in this gene are unknown. See McDermott et al [2022].


The m.7444G>A pathogenic variant located on the boundary of MT-CO1 and MT-TS1 has been reported to be causative for nonsyndromic hearing loss and aminoglycoside-induced hearing loss.

From: Genetic Hearing Loss Overview

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