Van Der Hoeve-De Kleiyn'S syndrome as a rare cause of hearing loss - two case reports.

It is relatively rare, dominant form of syndrome characterized by combination of blue sclerae brittle banes and Hearing loss. One of the conditions may be absent. Two cases, one 22 year old mtde with UltUtgray selrae md the other a 25 year old female with blue sclerae presented to us with hearing loss. Family history of hearing toss and blue sclerae were positive.

Incidence of pharyngeal hypophysis in neonates a histologic study.

The presence of adenohypophysial tissue in the nasopharynx is no longer disputed. This study was performed in 50 neonatal cadavers subjected to medical autopsy within 6 hours of death. The aim was to study the incidence of extrasellar pituitary tissue in the nasopharynx and its various histologic cell types. The transpalatal approach was used to obtain the specimens. The specimens were stained with hematoxylin and eosin and periodic acid-Schiff-orange G for selective demonstration of adenohypophysial cells. Histopathologic evaluation led to the detection of pituitary tissue in 16% of the examined specimens. Selective staining demonstrated a 6% positive incidence of adenohypophysial cells. The pharyngeal hypophysis exists in 2 forms: a typical adenohypophysial collection of cells and an atypical subepithelial cluster. The incidence of hypophysial tissue was higher in the older neonates, perhaps because of hormonal stimulation of the caudal remnant of Rathke's pouch.

Audiovestibular profile in unilateral chronic suppurative otitis media.

AudioIogical tests including middle ear impedance test and vestibular function tests were administered to seventyfive patients of unilateral chronic suppurative otitis media. These patients were selected so as to exclude the influence of hereditory, drugs, systemic illness and the factors of aging. The subjects were subdivided into 3 groups : group 1 cases with central perforation; group 2 with marginal and group 3 with attic perforation respectively. On pure tone audiometry, 19 diseased ears (25.3%) showed sensorineural hearing loss of 40 dB or more at 4 KHz and 8 KHz. The difference in the mean bone conduction threshold of diseased and the nondiseased ears was statistically significant at 4 KHz and above. Results of specialised tests of hearing suggested cochlear pathology being responsible for sensorneural hearing loss. Electronystagmographic recording of saccades and bithermal caloric induced nystagmus revealed canal paresis on the diseased side in 4 patients (5.3%). These 4 patients had 15 years history of otorrhoea.

Secondary fungal infections in chronic suppurative otitis media.

A clinical, bacteriological and mycological study of 103 cases of 'safe' chronic suppurative otitis media with intractable otorrhoea was conducted to observe the role of fungi in the pathogenesis and perpetuation of the disease process. Mycotic otitis media was found in 42 cases (40.77%).45 patients with draining ears which grew pure bacterial growth were treated with ear drops. Fifteen of these patients were treated with topical antibiotics (polymyxin, Neomycin & Gramicidin). Other 15 patients were put on antibiotic steroid (Polymxin, Neomycin and Hydrocortisone) ear drops. The remaining 15 patients acted as controls with dry mopping treatment only.It was noted that prolonged exposure to antibiotic ear drops and antibiotic-steroid ear drops led to the growth of pathogenic fungi in 5 patients (33.33%) each from two study subgroups while the third control subgroup of 15 patients grew fungi of low virulence in 3 cases (20%).It was concluded that the routine and prolonged use of topical antibiotics or antibiotics-steroids in cases of wet 'safe' chronic suppurative otitis media, from the onset is not justified.

Rhabdomyosarcoma of head and neck--an analysis of 24 cases.

Clinical records of 24 cases or rhabdomyosarcoma involving head and neck region were studied. Majority of the patients (83.3%) were under five years of age. The common sites of involvement were the middle ear (50%) and the nasopharynx (17%). Embryonal variety was noted in majority of patients (83.3%). Pain and swelling in the post aural or pre-auricular region was the commonest mode of presentation. Last four cranial nerves were involved in eight patients and four patients had extension of the tumor in the posterior cranial fossa. Twenty patients having embryonal rhabdomyosarcoma received combination of chemotherapy and radiotherapy. Half of these remained tumor free upto six months. Surgical extirpation was done only in two patients, where the disease was localised. The tumor in these two patients was of pleomorphic variety and did not recur till two years after surgery.

A study of P300 in melancholic depression--correlation with psychotic features.

The relationship between delusions and hallucinations (psychotic features) in melancholic depression and auditory P300 was studied. Forty patients with melancholic depression as per DSM-III-R were studied, 20 of whom had hallucinations and/or delusions. The 20 patients of the two groups were individually matched on age, gender, sociodemographic variables, and psychopathology (except for psychotic features). Auditory P300 obtained by the "oddball paradigm" showed that those with hallucinations and/or delusions had a significantly smaller P3 amplitude. Significant correlations between "psychotic features" and P3 amplitude was found. Implications of these findings on research, classification, and management of depression have been outlined.

Electrical response audiometry (ERA) in infants.

The electric response audiometry is a useful diagnostic technique for identifying hearing loss in infants. Among the various electrical responses, the BSER enables us to arrive at an accurate early clinical diagnosis of hearing loss in infants so that effective auditory input, which is the prime requisite for the conceptual foundation for the growth of communication, can be provided through appropriate intervention programmes. The electric response audiometry provides information regarding the physiologic state of the peripheral organs and auditory pathways but however, cannot ascertain whether the infant can hear, in terms of perceiving auditory information. Inspite of this major limitation, the growing acceptance of brainstem electrical potentials in clinics and laboratories throughout the world attests to their increasing importance especially in the assessment of hearing sensitivity of infants.

Post traumatic facial paralysis--a review.

A retrospective analysis of 23 cases of post-traumatic facial nerve injury has been done. Majority of the cases had a conductive or a mixed deafness. The Schirmer's test has been considered to be one of the main indicators to determine the site of injury. The mastoid segment of the facial nerve was found to be injured in 52% of the patients. The surgical approach in patients with post-traumatic facial nerve paralysis should be transmastoid. This can be combined with a middle fossa approach in patients with a positive Schirmer's test.

Effect of instant preparation of betel nut (pan masala) on the oral mucosa of albino rats.

Paste made out of an instant betel nut preparation was painted into the oral cavity of 21 albino rats on alternate days for a period of six months. Biopsies were taken from the oral mucosa at the beginning of the study and every two months there after and compared histopathologically with those obtained from a control group of 14 albino rats. Mild to moderate loss of nuclear polarity and increase in keratoses, parakeratoses, inflammatory cell infiltration and vascularity were noted when compared to the control group. The increase in mitotic figures was statistically insignificant and no definite changes in pigmentation or atypical cells were seen. These findings suggest the possibility of mild leukoplakia. Submucosal collagen increased steeply and steadily throughout the study period and at the end of six months, 88.23 per cent of biopsies showed thickened and condensed sub-mucosal collagen, indicating submucous fibrosis.

Audiovestibular functions in rheumatoid arthritis.

Audiologic, middle ear impedance and vestibular function tests were administered to 25 patients with classical or definite rheumatoid arthritis and 25 normal control subjects of 30 to 50 years age group. Any possible influence of other common causes of hearing loss was avoided by carefully selecting the patients. Six patients had mild sensorineural hearing loss and another had bilateral mixed hearing loss. Results of tone decay test, speech reception threshold, speech discrimination score and short increment sensitivity index tests suggested cochlear pathology being responsible for the sensorineural hearing loss. There was no statistical correlation between hearing loss or otoadmittance abnormality and duration or activity of rheumatoid arthritis or positivity of rheumatoid factor. Electronystagmographic recording of saccades and bithermal caloric induced nystagmus revealed no significant abnormality.

Post laryngectomy complications and their mode of management--an analysis of 203 cases.

Two hundred and three laryngectomized patients have been evaluated for the possible causes of post operative complications. 58 out of 203 patients (28.5%) developed pharyngocutaneous fistula. Wound infection was present in 57 cases (28%). Two patients had flap necrosis and 12 patients developed dysphagia after surgery due to pharyngeal stenosis. The possible causes attributed to high occurrence of pharyngocutaneous fistula are, advanced nature of disease, preoperative radio therapy and wound sepsis. Prior to 1980, the incidence of pharyngocutaneous fistula in our series (Arora et al. 1983) was 41.6 percent. But it decreased to 13.7 percent between January 1981 to June 1987. The important factors which led to the decreased incidence of pharyngocutaneous fistula were thought to be the change of suture material for pharyngeal repair from chromic catgut to Vicryl, use of vacuum suction as a replacement of corrugated rubber drain and aseptic syringe drainage and postoperative coverage with broad spectrum antibiotics.

Computed tomography for determining pathways of extension and a staging and treatment system for juvenile angiofibromas.

Computed tomography (CT) has changed the preoperative assessment protocol of juvenile angiofibromas as it shows the extent of the tumor and its pathways of extension. Hence, operation can be done sooner and with minimal blood loss. Using CT scanning in 22 patients with nasopharyngeal angiofibromas, we found different pathways of extension of the tumor. We report a new staging system and surgical approaches, based on the stage of the tumor.

Auditory investigations in hypothyroidism.

Twenty patients with thyroprivic hypothyroidism with a mean age of 34.8 years (range: 15-50 years) were investigated. Peripheral/central functions of the auditory system were assessed in the hypothyroid state and they were re-evaluated in the euthyroid state after treatment with levothyroxine. The mean observation period for re-evaluation was 3.7 months (range 3-7 months). Sixteen patients with hypothyroidism (80%) demonstrated hearing loss when compared with randomly selected age- and sex-matched normal subjects. Twelve of the hypothyroid patients had sensorineural hearing loss and 4 had mixed loss. Special hearing tests revealed a cochlear type of hearing loss. The tympanogram curve was of Type B in one ear of each of the 4 patients. Brainstem electric response audiometry showed prolonged, absolute latency of wave V and interpeak latencies I-III and I-V. The amplitudes of waves I, II and V were reduced. Following treatment with levothyroxine a statistically significant improvement in hearing thresholds was observed by pure-tone audiometry. Tympanogram curves returned to normal in 3 out of 4 patients. Brainstem electric response parameters did not show significant reversibility to normalcy following treatment. The results of auditory investigations suggest a causal relationship between hypothyroidism and hearing loss. The site of lesion in the auditory system is probably at several levels, viz. in the middle ear, and at cochlear and retrocochlear sites.