Otolaryngological manifestation of Gorlin Goltz syndrome.

A 46-year-old Jordanian man was seen in ENT clinic complaining of progressive left nasal obstruction and hearing loss for the last five years with a history of multiple surgical removal of dental cysts. Clinical and radiological investigation revealed a cystic mass occupying the left maxillary sinus protruding to the nasal cavity, calcification of the falx cerebri and bifid ribs. On these findings Gorlin-Goltz syndrome was confirmed. The case is presented and the literature reviewed.

The impact of social and environmental changes on allergic rhinitis among Saudi children. A clinical and allergological study.

Eighty four children with allergic rhinitis out of 316 consecutive cases were investigated, treated and followed-up for 2 years. Diagnostic skin testing were done with identified common allergens found in Saudi environment (2). Cat fur, Bermuda grass and mesquite were the most common allergens found. Symptoms of recurrent rhinosinusitis, otitis media with effusion, tonsils and adenoids infection were commonly noticed among them. The rapid change of environment in Saudi Arabia in the form of agricultural and other factors seem to play part in the increasing incidence of allergic manifestation in paediatric population. With antiallergic treatment and control of infection, most of these children responded well and only four children required surgery. The treatment of allergic disease saves a lot of children from unnecessary surgical procedures usually carried out on them.

Hearing impairment in association with distal renal tubular acidosis among Saudi children.

A follow-up of seven patients with the autosomal recessive inherited syndrome of distal renal tubular acidosis (RTA) and sensorineural hearing loss is described. Five patients were diagnosed as having primary distal renal tubular acidosis and rickets, four were found to have severe sensorineural hearing loss of over 80 dB: two of which are brothers. Two patients were diagnosed as having secondary distal renal acidosis due to a genetic disorder called osteopetrosis; they are brothers and their audiograms showed a mild conductive hearing loss of an average 35 dB bilaterally. All patients had growth retardation with improvement due to alkaline therapy but their hearing loss was not affected by the medication. The pedigrees of two families with half sibs showed the familial incidence for consanguineous marriage. Consanguinity was found to be positive in five out of the seven patients. The tribal tradition in Saudi Arabia fosters consanguineous marriages for cultural and social reasons and pre-arranged marriages are still seen.

Familial hereditary progressive sensorineural hearing loss among Saudi population.

Clinical and audiological studies of 234 Saudi patients from the central area of Saudi Arabia with progressive sensorineural hearing loss were carried out in Riyadh. One hundred and sixty-four came from 38 families, i.e. 70% of the total and 30% were sporadic cases. Consanguinity was found in 80.8%. Their hearing loss was characterized as being bilateral sensorineural, starting at 1 kHz frequency and gradually sloping the pure tone audiogram to severe and profound hearing loss at 8 kHz. Special tests for recruitment indicate hair cell lesions. Caloric tests were normal in 56 patients tested. No abnormal internal ears were found in a C.T. scan done for 84 cases. Serology for TORCH and non-TORCH agents were done for (124) selected cases. Syphilis was negative, however, positive immunoglobulins for cytomegalovirus, herpes simplex type 1 and 2, and toxoplasmosis were seen in 52 cases. All patients came from one area called Qassim where old customs and the tradition of consanguineous marriage were still practiced. Some of these families are now under molecular and cytogenetic study.