Neurological associations in auditory neuropathy spectrum disorder: Results from a tertiary hospital in South India

Anjali Lepcha, Reni K Chandran, Mathew Alexander, Ann Mary Agustine, K Thenmozhi, Achamma Balraj, Anjali Lepcha, Reni K Chandran, Mathew Alexander, Ann Mary Agustine, K Thenmozhi, Achamma Balraj

Abstract

Aims: To find out the prevalence and types of neurological abnormalities associated in auditory neuropathy spectrum disorder in a large tertiary referral center.

Settings and design: A prospective clinical study was conducted on all patients diagnosed with auditory neuropathy spectrum disorder in the ear, nose, and throat (ENT) and neurology departments during a 17-month period. Patients with neurological abnormalities on history and examination were further assessed by a neurologist to determine the type of disorder present.

Results: The frequency of auditory neuropathy spectrum disorder was 1.12%. Sixty percent were found to have neurological involvement. This included cerebral palsy in children, peripheral neuropathy (PN), spinocerebellar ataxia, hereditary motor-sensory neuropathy, spastic paresis, and ponto-bulbar palsy. Neurological lesions did not present simultaneously with hearing loss in most patients. Sixty-six percent of patients with auditory neuropathy spectrum disorder were born of consanguineous marriages.

Conclusions: There is a high prevalence of neurological lesions in auditory neuropathy spectrum disorder which has to be kept in mind while evaluating such patients. Follow-up and counselling regarding the appearance of neuropathies is therefore important in such patients. A hereditary etiology is indicated in a majority of cases of auditory neuropathy spectrum disorder.

Keywords: Auditory neuropathy; Charcot- marie-tooth disease; Friedreich ataxia; Refsum disease; consanguinity; genetic counseling; hereditary sensory; motor neuropathy; neurologic dysfunction; sensorineural hearing loss.

Conflict of interest statement

Conflict of Interest: None declared.

Figures

Figure 1
Figure 1
Flow chart showing distribution of patients with ANSD into different groups, ANSD = Auditory neuropathy spectrum disorder
Figure 2
Figure 2
Visual evoked potential tracings showing bilateral anterior visual pathway dysfunction
Figure 3
Figure 3
Posterior tibial somato-sensory evoked potential of a patient revealing bilateral central pathway dysfunction

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