MedGen

An inherited or acquired condition characterized by the inability to hear in one or both ears. [from NCI]

A decreased magnitude of the sensory perception of sound. [from HPO]

Hidrotic ectodermal dysplasia 2, or Clouston syndrome (referred to as HED2 throughout this GeneReview) is characterized by a triad of major clinical features including partial-to-complete alopecia, nail dystrophy, and palmoplantar hyperkeratosis. Sweating is preserved and there are usually no dental anomalies. Sparse scalp hair and dysplastic nails are seen early in life. In infancy, scalp hair is fine, sparse, and brittle. Progressive hair loss may lead to total alopecia by puberty. The nails may be milky white in early childhood; they gradually become dystrophic, thick, and distally separated from the nail bed. Palmoplantar keratoderma may develop during childhood and increases in severity with age. Associated features may include cutaneous hyperpigmentation (particularly over the joints) and finger clubbing. The clinical manifestations are highly variable even within the same family. [from GeneReviews]

A partial or complete loss of hearing in one or both ears. It is classified as conductive, sensory, or central. [from NCI]

Progressive loss of neural cells and tissue. [from HPO]

A non-neoplastic or neoplastic disorder that affects the ear. Representative examples include infections, hearing disorders, benign neoplasms, and carcinomas. [from NCI]

Lack of measurable response to stimulation of auditory evoked potentials. [from HPO]

A tumor (abnormal growth of tissue) of the inner ear. [from HPO]

A benign or malignant neoplasm affecting the eighth cranial nerve, also referred to as the vestibulocochlear nerve. Clinical features may include hearing loss, vertigo, tinnitus, headache and involvement of the trigeminal or facial nerves. [from NCI]