Hereditary Sensorimotor Neuropathy with Sensorineural Deafness(CMT4D ): A Case Report | 2004, Volume 10 - Issue 5

Özgür BİLGİN¹, İpek MİDİ¹, Geysu KARLIKAYA², Neşe TUNCER ELMACI¹, Tülin TANRIDAĞ¹

¹Marmara Üniversitesi Tıp Fakültesi Nöroloji Anabilim Dalı, İSTANBUL
²Haydarpaşa Numune Hastanesi 2. Nöroloji Kliniği, İSTANBUL

Keywords: hereditery sensorimotor neuropathy,Sensorimotor deafness

Abstract

Scientific background: Hereditary sensorimotor neuropathies(HSMN) are disorders of peripheral nerves with predominantly schwann cell and myelin involvement in which prevalence is 1/3300, most common among the genetic neurological disorders. The association of HSMN and sensorineural deafness is rare. Case: 25 year-old woman with HSMN and sensorineural deafness is presented in whom symptoms began in the first 10 years and evolved in slowly progressive fashion. Conclusion: The aim of this case report is to demonstrate an assosiation of HSMN and sensorineural deafness which is quite rare in the literature