Meniere's syndrome, otosclerosis, and insulin resistance syndrome.

A 45-year-old man presented to the author's office with a long-standing history of dizziness. His otologic symptoms had begun when he was approximately 13 years old; at that time, he first became aware of tinnitus in both ears in relative quiet while hunting. A decade later, a physician had recommended that he consider amplification to enhance his hearing, which had begun to deteriorate. But the patient declined to use a hearing aid, and his bilateral tinnitus and hearing loss progressed. When he was about 30 years of age, he experienced a brief spell of light-headedness with some nausea. These symptoms recurred about twice a year, but the patient shrugged them off as unimportant.

Ten months prior to presentation, the patient underwent a change in ear symptoms, as he began to experience continuous aural fullness and corresponding hearing loss on the right. The combination of the increase in aural fullness and tinnitus produced a pulsating sound that had been present ever since. The loudness of the sound increased as the fullness increased. The intensity of the fullness would fluctuate; it tended to be strongest just prior to a dizzy spell. The patient likened his condition during the dizzy spell to "being drunk." He was unable to walk, he experienced diaphoresis, nausea, and sometimes vomiting, and he felt that he needed "to sleep it off." These spells lasted for a couple of hours. Afterward, the patient would be fatigued and "in a tog" for several days. These attacks occurred as often as two or three times per week.

At that point, the patient sought treatment from another physician. The physician prescribed a diuretic, which seemed to help for a few weeks. Later, diazepam was added to the regimen, and it too seemed to help for a couple of weeks. Six weeks prior to presentation to the author, the patient underwent treatment with the Meniett low-pressure pulse generator as a prelude to gentamicin injection. Use of the Meniett device alleviated his symptoms markedly, but his condition deteriorated following a deep massage. The adverse effect of the massage lasted for a couple of weeks before the patient began to feel better.

In the author's office, the patient reported that the hearing in his right ear continued to worsen as the aural fullness increased. In his left ear, the tinnitus had stabilized and there was no further progression of hearing loss. He continued to take hydrochlorothiazide and diazepam.

Physical examination revealed that the patient had a pressure-equalizing tube in the right ear. He had difficulty performing the sharpened tandem Romberg test with the right foot forward. A tuning-fork test indicated that the hearing loss on the right was severe. Audiometry confirmed the flat severe sensorineural hearing loss on the right and documented a speech reception threshold of 80 dB and a speech discrimination score of 24%. Contralateral acoustic stapedial reflexes identified a cochlear site for the bearing loss. Electronystagmography revealed no spontaneous or positional nystagmus. The alternate binaural bithermal test revealed a 68% reduced vestibular response (RVR) on the right with no significant directional preponderance, and the simultaneous binaural bithermal test showed a type 2 response, confirming the RVR on the right.

Thin-section, small-pixel imaging of the temporal bones demonstrated findings consistent with inner ear otosclerosis. A metabolic evaluation revealed a markedly elevated serum triglyceride level (258 mg/dl). On the 5-hour glucose tolerance test, the serum insulin level reached 140 [micro]U/ml at the first hour and 123 [micro]U/ml at the second hour, findings consistent with insulin resistance. The diazepam and hydrochlorothiazide were discontinued, and the patient was placed on a diet to address his metabolic factors. For the otosclerosis, he was prescribed risedronate at 30 mg twice weekly along with daily supplemental calcium, fluoride, and vitamin D.

The patient returned 3 months later and reported that he had experienced two bouts of mild dizziness during the day and at times at night. His hearing was subjectively the same, but audiometry in the right ear showed improvements in his pure-tone averages (PTAs), speech reception threshold (60 dB), and speech discrimination score (76%). The risedronate was suspended for 2 weeks every 6 weeks and replaced with etidronate at 400 mg/day, and the dietary plan was extended.

The patient returned 3 months later and reported no new episodes of dizziness. He was not sure if his hearing was better, but his tinnitus on the right had diminished to the point that the pulsating sound was minor and the ringing was faint. There was no aural fullness. The PTAs on the right were the same, his speech reception threshold continued to improve, and his speech discrimination score had dropped to 60%. The drug regimen was continued at 8-week intervals.

This patient's otologic history began early in life with bilateral sensorineural hearing loss and tinnitus that segued into dizziness and eventually symptoms of Meniere's syndrome in the right ear. Conventional medical management and use of the Meniett device failed to control his symptoms. Audiometry and electronystagmography identified the source of the symptoms as the right labyrinth. The etiologic evaluation identified the cause as inner ear otosclerosis and insulin resistance syndrome. Treatment directed at the etiology brought the symptoms under control.

Kenneth H. Brookler, MD

From Neurotologic Associates, PC, New York City.