ABSTRACT
Fibrous dysplasia is a fairly common, localized misdifferentiation of the bone-forming mesenchyme affecting a single or many bones, in which skeletal aberrations represent the cardinal feature, but in which certain endocrinopathies, abnormal pigmentation of skin and mucous membrane, and occasionally other abnormalities form part of the entire disease process. The craniofacial skeleton is one of its predilective sites and therefore the temporal bone may become involved. In such instances the disease manifests itself with 1) progressive loss of hearing, 2) increasing obliteration of the external ear canal, and 3) enlargement and distortion of the temporal bone. The first part of this communication is concerned with a review of the clinical and pathological aspects of the disease in general. The second part is concerned with a discussion of the clinical manifestations in the temporal bone, based upon analysis of the entire literature and the authors' personal experience. The indications for surgical intervention include 1) maintenance of a normal outer ear canal, 2) preservation of cochlear and vestibular function, and 3) prevention of secondary complications.
Subject(s)
Fibrous Dysplasia of Bone/pathology , Temporal Bone/pathology , Adolescent , Adult , Age Factors , Bone and Bones/pathology , Cell Transformation, Neoplastic/pathology , Child , Child, Preschool , Diagnosis, Differential , Female , Fibrous Dysplasia of Bone/diagnosis , Fibrous Dysplasia of Bone/diagnostic imaging , Fibrous Dysplasia, Monostotic/pathology , Fibrous Dysplasia, Polyostotic/pathology , Hearing Disorders/etiology , Humans , Male , Middle Aged , Nervous System/physiopathology , Prognosis , Radiography , Sex Factors , Skin/pathology , Skin PigmentationABSTRACT
A previously undescribed syndrome with profound sensorineural hearing loss since early infancy, episodes of dizziness, and hypodontia developed in a brother and sister. The presence of two unaffected children and unaffected parents suggests autosomal recessive inheritance.
Subject(s)
Anodontia/genetics , Hearing Disorders/genetics , Vertigo/genetics , Child , Child, Preschool , Female , Humans , Male , SyndromeABSTRACT
A hitherto unreported treatment for one of the manifestations of the hyoid syndrome is excision of the tip of the hyoid greater cornu. A greater "index of suspicion" for the hyoid syndrome is urged.