Table 4

Neurological signs in 66 TAN patients

Signs n %
Cognitive impairment11.5
Bilateral optic atrophy3248.5
Facial nerve palsy34.5
Bilateral neurosensory deafness1116.0
Conductive deafness11.5
Motor wasting
 Small muscles of hands1522.7
 Quadriceps and calves69.0
Abnormal tone00.0
Normal muscle power66100.0
Loss of deep reflexes
Increased deep reflexes
 Finger flexion23.0
 Knee jerk3959.1
 Ankle jerk00.0
Superficial reflexes
 Loss of abdominal6091.0
 Loss of plantar2441.0
 Extensor plantar23.0
Primitive reflexes
 Orbicularis oris710.6
Sensory gait ataxia4873.0
Positive Romberg test1827.3
Sensory loss
 Pin prick66100.0
 Cotton wool6395.5
 Joint position sense1929.0
 Vibration sense3959.1