Syndrome of recurrent meninigitis due to congenital perilymph fistula with two different clinical presentations.

Recurrent meningitis secondary to a congenital labyrinthine anomaly is a rare clinical entity, diagnosis of which is dependent upon certain clinical, radiological and intraoperative features. In the following report we describe two children with congenital labyrinthine fistula and recurrent meningitis whose clinical presentation, radiological features and intraoperative findings were dissimilar and thus, illustrative of two different ways of presentation of this rare disorder. While one had a classical Mondini defect and unilateral hearing loss, the other had normal audiometric and radiographic findings. The fistulae were successfully closed via a tympanotomy approach in both the patients.

Pediatric nocturnal enuresis secondary to airway obstruction from cleft palate repair.

Nocturnal enuresis in children can at times be alleviated or resolved completely with surgery on the upper airway. We present a report of a 7-year-old boy in whom nocturnal enuresis began immediately after reconstructive surgery of the pharynx that caused upper airway obstruction. Enuresis diminished immediately and stopped following an adenoidectomy but resumed secondary to adenoid regrowth after more than a year of dry nights. Nocturnal enuresis was immediately and permanently relieved by adenoidectomy revision. We review the relevant literature and discuss possible physiologic relationships between upper airway obstruction and bladder and renal function.

Inferior turbinate reduction surgery in children.

We assessed the effectiveness and safety of surgical reduction of the inferior turbinate in 64 children, ages three to 15 years, who were operated on between January 1985 and November 1995. All patients/parents were interviewed and assessed by means of a questionnaire one to 10 years postoperatively. All patients had follow-up and were evaluated clinically at one and two weeks postoperatively. Long-term follow-up visits occurred at six, 12 and 24 months. Eighty-nine percent of the children/parents reported a sustained improvement in their nasal airways. No cases of serious postoperative hemorrhage, excessive dryness nor long-term crusting of the nose were observed or reported. We conclude that in the treatment of children with severe nasal obstruction, surgical reduction of the inferior turbinate is a safe and effective operation, alone or in combination with other airway procedures, and may increase the effectiveness of subsequent medical management.

Benign paroxysmal positional vertigo: analysis of 44 cases treated by the canalith repositioning procedure of Epley.

Between September 1987 and February 1993, the authors treated 44 consecutive cases of benign paroxysmal positional vertigo with the canalith repositioning procedure of Epley. The authors report a success rate of 87.7 percent using this treatment technique. Clinical observations and case reports are presented.

Geniculate ganglion: anatomic study with surgical implications.

The geniculate ganglion and adjacent segments of the facial nerve were dissected in 11 human temporal bones to study the extent and distribution of ganglion cells. A histologic basis for the use of geniculate ganglionectomy as the treatment for geniculate neuralgia was sought. In 9 of 11 specimens (81.8%), the ganglion cell bodies appeared to be aggregated at the apex of the genu close to the origin of the greater superficial petrosal nerve. The mean ratio of the width of the ganglion cell cluster to the width of the facial nerve trunk at the level of the genu was 0.4. In two specimens, significant anatomic variation was present. One specimen showed extension of cell bodies into the labyrinthine segment of the facial nerve; another specimen showed a single ganglion cell in the region of the genu. These findings lead us to postulate that geniculate ganglionectomy may be ineffective as the sole treatment for certain cases of geniculate neuralgia, and that nervus intermedius section may also be required to achieve a more complete deafferentation.

Treatment and management of perilymphatic fistula: a New Hampshire experience.

From August of 1975 to June of 1990 the author managed 45 cases of spontaneously occurring perilymphatic fistula. Twenty-nine of the 45 cases were reported in 1988. In the series there were eight patients with bilateral involvement, seven with involvement of both oval and round window in the same ear, and seven with fistulas believed to be of congenital origin. Seventeen patients (38%) required revision surgery. Occasionally adjunct modalities (endolymphatic shunt, labyrinthectomy, blocking a cochlear aqueduct, and streptomycin ablation) were used for symptom control. Diagnosis, treatment, and management of these patients is discussed.

Geniculate neuralgia: the surgical management of primary otalgia.

Intractable, unexplained deep-ear pain presents a rare, albeit significant problem in otolaryngological and neurosurgical practice. The authors review their experience with 18 cases of primary otalgia during the past 15 years. A total of 31 surgical procedures were performed. Seventeen patients had sequential rhizotomies and one patient had microvascular decompression alone. Based on the clinical diagnosis, the nerves sectioned were singly or in combination: the nervus intermedius (14 patients), geniculate ganglion (10 patients), ninth nerve (14 patients), 10th nerve (11 patients), tympanic nerve (four patients), and chorda tympani nerve (one patient). Microvascular decompression of the involved nerves was undertaken in nine patients, in whom vascular loops were discovered. Adhesions (six patients), thickened arachnoid (three patients), and benign osteoma (one patient) were other intraoperative abnormalities noted. The overall success of these procedures in providing pain relief was 72.2%, and the mean follow-up period was 3.3 years (range 1 month to 14.5 years). There was no surgical mortality. Expected side effects were: decreased lacrimation, salivation, and taste related to nervus intermedius nerve section, and transient hoarseness and diminished gag related to ninth and 10th nerve section. Four patients developed sequelae consisting of sensorineural hearing loss, vertigo, and transient facial nerve paresis. One patient had a cerebrospinal fluid leak and another developed aseptic meningitis as postoperative complications. Except when primary glossopharyngeal neuralgia is the working diagnosis, a combined posterior cranial fossa-middle cranial fossa approach is recommended for adequate exploration and/or section of the fifth, ninth, and 10th cranial nerves as well as the geniculate ganglion and nervus intermedius.

Nocturnal enuresis in children with upper airway obstruction.

This study presents the results of our experience with 115 children between the ages of 3 and 19 years who have had symptoms of upper airway obstruction and nocturnal enuresis. Twelve children had secondary enuresis, and 103 children had primary enuresis. Surgical removal of upper airway obstruction led to a significant decrease in or complete cure of nocturnal enuresis in 87 (76%) of the children studied. Eleven children were also studied with polysomnographic tracings in an attempt to determine a relationship between their sleep patterns and nocturnal enuresis.

Sensori-neural hearing loss in acute otitis media due to beta-hemolytic streptococcus successfully treated with penicillin and prednisone.

This article reports a case of a 21-year-old woman with acute otitis media with effusion secondary to a virulent group A beta-hemolytic streptococcus infection that led to rapidly deteriorating hearing in an only hearing ear. This deterioration continued despite middle ear ventilation and high-dose intravenous cefazolin and subsequent high-dose aqueous penicillin. The addition of prednisone to her therapeutic regimen led to rapid hearing improvement and decrease in effusion production. A historical perspective on the use of steroids in treating otitis media and several theories for this reversal in clinical course are presented.

Repair of a cerebrospinal fluid perilymph fistula primarily through the middle ear and secondarily by occluding the cochlear aqueduct.

A 35-year-old man had a 5-year history of fluctuating hearing loss in his only hearing ear. History and diagnostic tests indicated a perilymph fistula, a diagnosis subsequently confirmed by exploration. Primary and secondary repairs temporarily ameliorated symptoms. A spinal fluid to middle ear fluid pathway was identified by radioactive tracer. A patent cochlear aqueduct indicated on computed tomography scan was found and repaired through a posterior cranial fossa approach. Hearing was preserved, remaining relatively stable during the 2-year follow-up period.

Objective tinnitus of vascular origin with hearing improvement after treatment.

Objective tinnitus occurs as a result of muscular contraction or vascular pulsations. The literature is reviewed and we present two case reports of vascular objective tinnitus and its diagnostic and therapeutic modalities. One patient was treated with embolization alone; the other, with embolization and surgical ligation. Both patients achieved an improvement in sensorineural hearing thresholds in their affected ears; one patient experienced an improvement in speech reception threshold of 30 decibels. Measures useful to diagnosis and evaluation of therapy and reasons for the observed improved hearing thresholds are discussed.

Perilymphatic fistula: a New Hampshire experience.

Thirty-five patients with perilymphatic fistulas (PLFs) are presented. Of this group of 35 patients (39 ears), 4 patients did not have fistulas that could be observed with certainty but were presumed to have fistulas by virtue of their excellent response to surgical repair. Our case reports provide examples of the great variety and possible classifications of presentations and symptom complexes that lead one to suspect the diagnosis of perilymphatic fistula. Comments on diagnostic and therapeutic modalities and on postoperative care and counseling are included. The age range of patients in our series is 3 to 67 years. Four patients are under age 20, and an additional three patients probably developed their symptoms prior to age 20 but presented later. Twenty-three (79%) of 29 patients with spontaneous PLFs began having symptoms closely related to some event involving physical or mechanical stress, and a high percentage (76%) had symptoms aggravated by physical stress. Six are believed to have fistulas of congenital origin. There is a sibling pair and a mother and son in the series; these four people had bilateral fistulas.

Spontaneous cerebrospinal fluid otorhinorrhea.

Spontaneous cerebrospinal fluid (CSF) otorrhea implies the observation of CSF draining from the ear or nose without any previous history of traumatic or infectious etiology. The term does not exclude embryologic or developmental abnormalities. The condition may be life-threatening. Thus far it has been reported about twenty times. Because it is rare, misdiagnosis or failure to make a timely early diagnosis is common and proper therapy may be delayed. The purpose of this article is to review pertinent literature on this subject and to present two additional case reports. Emphasis will be placed on accuracy of diagnosis and on the surgical technique employed in repair of the discovered defect.

Nocturnal enuresis in children with upper airway obstruction.

This article reviews 35 children between the ages of 3 1/2 and 11 years who have had symptoms of upper airway obstruction and enuresis. Twenty-eight patients had primary enuresis, and 7 had secondary enuresis. Removal of upper airway obstruction by surgical intervention led to a significant decrease or complete cure of nocturnal enuresis in 26 patients. Four children were studied with polysomnographic tracings in relationship to their enuresis.

Computed tomography of the ossicles.

Otologists and otolaryngologists have described in detail the disorders which are unique to the ossicles. However the anatomy and spectrum of pathology of the ossicles are not familiar to most radiologists. Recent advances in computed tomography (CT) and a systematic approach to evaluation now allow accurate identification of even subtle abnormalities of the ossicles. We present the normal anatomy, ossicular abnormalities, and indications for computed tomographic study. Because of the greater diagnostic capability of CT, the radiologist's role has increased in evaluation and treatment planning of patients with suspected ossicular abnormalities.

Past, present, and future applications of the auditory middle latency response.

For many years (auditory) middle latency response (MLR) reportedly has been an excellent electrophysiological procedure for estimating frequency specific auditory thresholds. Currently the 40 Hz potential, a derivative of the MLR, holds even greater promise for threshold testing. MLR also appears to have potentially valuable applications in the evaluation of functional hearing loss and assessment of higher auditory function. However, MLR is not without controversy. Questions of origin, filtering effects, and its use in pediatric assessment are current issues surrounding applications of MLR. The following article analyzes the values and limitations of MLR within the framework of past, present, and future research and clinical uses.

Bilateral congenital oval window microfistulae in a mother and son.

This article details case histories of a son and his mother who were studied over a seven-year period before making the diagnosis of bilateral oval window microfistulae in both of them. The boy demonstrated a slowly progressive bilateral and symmetrical sensorineural hearing loss closely resembling that of his mother. The case reports emphasize their respective histories, diagnostic evaluation, surgical observations, and treatment.

Laryngomalacia. A review and case report of surgical treatment with resolution of pectus excavatum.

Laryngomalacia has been described in the medical literature for over 100 years. During this time many different names have been used for the same condition. There have been numerous discussions of symptoms, cause, pathogenesis, relationships to other conditions, hereditary factors, and treatment. The purpose of this study is to historically review the literature, clarify terminology, and describe the clinical aspects of the disease. We also summarize and make conclusions regarding cause, pathogenesis, genetic and disease relationships, prognosis, and treatment. Finally, we present a case report of severe laryngomalacia and describe a surgical technique used to treat it.