Otosclerosis is a disease that has seen its share of controversy over the past 40 years. It now represents a readily diagnosable and treatable process in which the surgical techniques for correction with some variability have been nearly perfected. Even now, however, some areas of controversy remain in the management of otosclerosis, specifically in patient selection for therapy, in treatment options, and in surgical techniques. Controversies in patient selection include determining the candidacy of groups that have unique issues that define the limits of intervention, such as children, the elderly, aviators and pilots, patients with small air–bone gaps, and patients with coexisting endolymphatic hydrops. Other issues include selecting patients for revision stapedectomy and anticipating problems that can be encountered in these cases. Differing opinions exist with regard to prosthesis choice, use or nonuse of tissue grafts, laser instrumentation, management of a mobile footplate, type of anesthesia, and amount of footplate removed. Finally, for some the medical management of otosclerosis and the use of fluoride or biphosphonates remains controversial. We present a discussion of these topics and our management of otosclerosis.

Background

Controversy in the management of otosclerosis began in 1876 with the first attempt at surgery for otosclerosis with total stapedectomy by Kessel. Unsuccessful largely due to a lack of sterile technique and antibiotics, otosclerosis surgery was abandoned until 1923, when Holmgren performed a three-stage lateral semicircular canal fenestration, a technique later improved by Lempert. The next era of controversy paralleled advances in surgical technique. Very controversial at the time, the first modern successful stapedectomy technique was performed and described by Shea in 1956.^{1, 2}

Several modifications of the technique and prostheses have since evolved, with the overall success rates (four frequency average of conductive gap closure within 10 dB) in experienced hands climbing to more than 90%. Laser applications have added the ability to achieve hemostatic and atraumatic access to the footplate/oval window interface. This has become most useful during revision procedures when working with unknown footplate status, mucosal scarring at the oval window (OW) and the potential of intralabyrinthine adhesions. In recent series, laser revision techniques have demonstrated improved results, due to this ability to open the OW seal atraumatically and allow the surgeon to reestablish ossicular chain continuity.^3–6 Before laser revision techniques, reopening of the OW was associated with a high rate of sensorineural hearing loss (SNHL) and was avoided. Lasers, of both visible and nonvisible wavelengths, have been shown to be safe and effective in primary and revision stapes surgery. With all laser types performing equally well, choice is determined by cost, availability, and delivery system preference.^3–10

Discussion

INDICATIONS FOR PATIENT SELECTION

Bone conduction greater than air (negative Rinne), using a 512-Hz tuning fork with appropriate masking, should be reproducible on at least two separate occasions. Audiometric data should demonstrate a predominant low-frequency conductive gap that averages ࣙ10 dB in the speech frequencies, which may narrow with a mild sensorineural notch at 2000 Hz (Carhart notch). Acoustic reflexes must be absent with normal compliance tympanometry. Ideally, speech discrimination scores (SDS) should be greater than 80%, unless the loss is severe. Patients with poor SDS may have other neural disease, including cochlear otosclerosis, portending a suboptimal surgical result.

INDICATIONS BY AGE

Age is not a contraindication to surgery. If the individual is in reasonable health, both the elderly and children can be appropriate candidates for surgery. The elderly can tolerate local anesthesia without difficulty when counseled appropriately. In our experience with 154 patients ranging in age from 70 to 92 years, these patients were as likely to obtain a successful hearing result (93%) with improvement in SDS and to avoid vestibular symptoms as were patients under age 70.¹¹ Studies have documented the safety of stapedectomy in children with appropriate evaluation.^{12, 13} Children older than 5 years of age who are not susceptible to otitis media do well with a general anesthetic. Surgery can be delayed in this group if otitis confuses the clinical picture or delayed until the child is older and mature enough to cooperate with local anesthesia. We are much more likely to delay surgery in unilateral losses. In our experience with 47 ears of children aged 17 or younger, successful results were obtained in 92%. These children were less likely to obtain overclosure and were three times more likely to require a drillout. Long-term results were stable with minimal decay.¹²

Patients with concomitant otosclerosis and Meniere’s disease can be difficult to manage. In patients with active Meniere’s disease, stapedectomy has a high rate of severe or total sensorineural hearing loss (SNHL). Some have attributed this to an enlarged saccular or Reissner’s membrane abutting the under surface of the footplate, which is traumatized at footplate fenestration. Although some otosclerotic patients have transient dysequilibrium made worse by sudden movements, few rarely describe rotatory vertigo. The patients who have episodic vertigo or other symptoms suggestive of Meniere’s disease should undergo a formal vestibular work-up and treatment as necessary prior to stapes surgery. Only patients who have had symptoms of Meniere’s disease inactive for several years, and with bone conduction levels of ࣙ35 dB at 500 Hz and no high-frequency loss, as well as good discrimination, should be considered for stapedectomy.14

AIRCREW AND COMBAT PILOTS

Aircrew and pilots with otosclerosis also pose an interesting dilemma. As it progresses, hearing loss is burdensome. However, performing a procedure that opens the OW may put them at risk of fistula and untimely vestibular symptoms, which would disqualify them from flight status. The Federal Aviation Administration (FAA) policy is to grant waivers from disqualification due to stapedectomy on an individual basis, pending clearance from the surgeon. Up to 1994, no waived commercial pilot has had any mishaps or complications from a stapedectomy; recent studies have also shown that stapedectomy in military flight team members can be safe and successful.^{15, 16} Data compiled from combat pilots in the Israeli Air Force have demonstrated that pilots undergoing stapedectomy with a large vein graft and Robinson prosthesis can safely return to combat-intensity flying without concern for malfunction of the stapedectomy. Nine pilots operated on by the senior author obtained successful hearing results and have safely logged more than 4160 hours of high-performance flying without vestibular symptoms. The senior author recommends only procedures using a vein or tissue graft and comprehensive postoperative vestibular and altitude chamber testing at 3 months, before approval for return to flight status.¹⁶

SMALL AIR–BONE GAPS

Because some otosclerotic patients retain a small conductive hearing loss for years, whereas others progress rapidly, many surgeons prefer to wait for a larger conductive loss to improve the risk/benefit ratio for stapedectomy. Current guidelines have been reported that advocate a 20-dB pure-tone average air– bone gap.¹⁷ However, for experienced stapedectomy surgeons with high rates of success and low rates of complication, a high rate of overclosure in recovering cochlear reserve has occasionally allowed surgery to correct air–bone gaps as small as 10 dB.¹⁸ In our experience with 136 patients, almost all had a family history or previous diagnosis of otosclerosis in the opposite ear. All had appropriate tuning fork tests and absent acoustic reflexes. Only if otosclerosis was surgically confirmed did the surgeon proceed with a stapedectomy. These patients had rejected hearing aids but desired improved hearing. Mean hearing improvement was 16.7 dB with mean overclosure of 8.1 dB. More than 89% of patients in that study overclosed their air–bone gap.¹⁸

INDICATIONS FOR REVISION STAPEDECTOMY

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