Annals of Indian Academy of Neurology
ORIGINAL ARTICLE
Year
: 2015  |  Volume : 18  |  Issue : 2  |  Page : 171--180

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


Anjali Lepcha1, Reni K Chandran2, Mathew Alexander3, Ann Mary Agustine1, K Thenmozhi1, Achamma Balraj1 
1 Department of Ear, Nose and Throat, Christian Medical College and Hospital, Vellore, Tamil Nadu, India
2 Department of Otolaryngology, Head and Neck Surgery, Hamad Medical Corporation, Al Wakra Hospital, Doha, India
3 Department of Neurology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India

Correspondence Address:
Anjali Lepcha
Department of Ear, Nose and Throat, Unit IV, Christian Medical College and Hospital, Vellore - 632 004, Tamil Nadu
India

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.


How to cite this article:
Lepcha A, Chandran RK, Alexander M, Agustine AM, Thenmozhi K, Balraj A. Neurological associations in auditory neuropathy spectrum disorder: Results from a tertiary hospital in South India.Ann Indian Acad Neurol 2015;18:171-180


How to cite this URL:
Lepcha A, Chandran RK, Alexander M, Agustine AM, Thenmozhi K, Balraj A. Neurological associations in auditory neuropathy spectrum disorder: Results from a tertiary hospital in South India. Ann Indian Acad Neurol [serial online] 2015 [cited 2019 Aug 19 ];18:171-180
Available from: http://www.annalsofian.org/article.asp?issn=0972-2327;year=2015;volume=18;issue=2;spage=171;epage=180;aulast=Lepcha;type=0