Objective: This case series aims to describe the diagnosis and management of otosclerosis in a series of Filipino patients encountered in a private, subspecialty otologic clinical practice. In particular, it elucidates the demographics, clinical presentation, diagnostic exam findings, clinical intervention and hearing outcomes. Methods: Study Design : Retrospective review of medical records of a case series. Setting : Subspecialty otologic / neurotologic clinical practice. Participants : Medical records of all patients diagnosed to have otosclerosis based on radiologic evidence of fenestral or retrofenestral otosclerosis and/or confirmed during surgical exploration of the middle ear, during the period 2004-2017. Results: Nine patients with otosclerosis were identified. Most patients presented with bilateral, primarily conductive hearing loss in middle age. However, an adolescent patient was also identified. Most patients were from the component cities of the National Capital Region. However, patients from the Cordillera region and south-central Mindanao were also identified. Radiologic evidence of fenestral or retrofenestral otosclerosis was identified in the majority of patients. However, readings of normal temporal bone CT findings were also seen. Obliterative otosclerosis was identified in one patient. Both objective and subjective evidence of hearing improvement was documented after stapedectomy in patients undergoing surgery. Conclusions This study documents the presence of clinical otosclerosis in a Filipino population. As in other populations, it typically presents in middle age as a bilateral, primarily conductive hearing loss. However, a younger age does not preclude its diagnosis, as juvenile otosclerosis has been identified. The presence of patients in regions of the Philippines other than the National Capital Region implies that it has to be considered even in regional populations without a racial predilection for otosclerosis. The identification of radiologic evidence of fenestral and retrofenestral otosclerosis on CT imaging of the temporal bone makes this is an essential part of the work-up of Filipino patients with conductive hearing loss and normal otologic examinations. Surgical treatment via stapes surgery is an effective and viable option in the management of Filipino patients with otosclerosis. The identification of less common variants such as obliterative otosclerosis indicates the need for specialized surgical equipment and appropriate surgical training in order to successfully deal with these situations.
Otosclerosis ; Hearing Loss, Conductive

Humans ; Otosclerosis/surgery* ; Stapes ; Stapes Surgery/adverse effects* ; Arteries

Humans ; Stapes ; Otosclerosis/surgery* ; Ossicular Prosthesis ; Stapes Surgery ; Incus

Objective: To analyze the surgical efficacy of patients with mixed hearing loss and otosclerosis with different air bone gap (ABG) before surgery, and to provide reference for the prognosis evaluation of otosclerosis surgery. Methods: The clinical data of 108 cases(116 ears) of otosclerosis who had undergone stapes fenestration technique artificial stapes implantation in Xiangya Hospital of Central South University from November 2013 to May 2020 and had mixed hearing loss before surgery were collected, including 71 women(76 ears)and 37 men (40 ears), with an average age of 38.5 years. According to preoperative pure tone audiometry ABG, they were divided into three groups: group S, 15 dB≤ABG<31 dB, a total of 39 ears; group M, 31 dB≤ABG<46 dB, a total of 58 ears; and group L, ABG≥46 dB, 19 ears in total. The hearing outcomes of three groups of patients at 6-12 months after surgery were compared and analyzed using SPSS 24.0 statistical software. Results: A total of 3 patients (group S: 2 cases; group L: 1 case) experienced severe sensorineural hearing loss after surgery and were not included in the statistical analysis. After surgery, the pure tone hearing threshold of patients with otosclerosis in each group was significantly improved compared to before surgery, with an average air conduction threshold improvement of(21.6±13.4) dB. The difference between before and after surgery was statistically significant(t=17.13, P<0.01). The average bone conduction threshold improved by(3.7±7.6) dB, and the difference was statistically significant before and after surgery(t=5.20, P<0.01). The postoperative ABG was(18.3±9.3) dB, which was significantly reduced compared to preoperative(36.2±8.6)dB. Among the three groups of patients, the L group had the highest improvement in air conduction threshold[(29.9±10.8)dB], while the S group had the lowest improvement[(15.7±11.4)dB]. There was no statistically significant difference in post operative pure tone hearing thresholds between the three groups(P>0.05). The postoperative ABG in group S was the smallest[(16.5±9.0)dB], while in group L, the postoperative ABG was the largest[(20.5±10.0)dB]. Compared with group S, group M and group L still had a large residual ABG at 2 000 Hz after surgery. The bone conduction threshold of both S and M groups improved to some extent after surgery compared to before (P<0.01). Conclusions: Surgery can benefit patients with mixed hearing loss and otosclerosis with different preoperative ABG. Patients with small preoperative ABG have better surgical results and ideal ABG closure at all frequencies after surgery. Patients with large preoperative ABG can significantly increase the gas conduction threshold during surgery, but certain frequencies of ABG may still be left behind after surgery. The improvement effect of surgery on bone conduction threshold is not significant. Patients should be informed of treatment methods such as hearing aids based on their actual situation for selection.
Male ; Humans ; Female ; Adult ; Bone Conduction ; Otosclerosis/surgery* ; Hearing Loss, Mixed Conductive-Sensorineural/surgery* ; Stapes Surgery/methods* ; Treatment Outcome ; Auditory Threshold ; Hearing ; Audiometry, Pure-Tone ; Deafness ; Retrospective Studies

A 29-year-old Filipina of Chinese descent presented with progressive bilateral conductive hearing loss of several years’ duration. While working overseas, she consulted with an otolaryngologist and underwent computerized tomographic (CT) imaging of the temporal bone as part of her evaluation. She was informed that no abnormalities were identified in the imaging exam, and she was offered exploratory middle ear surgery with possible stapes surgery. She then sought a second opinion, with the intention of obtaining a more definitive diagnosis prior to any invasive medical intervention. A review of the CT imaging study, with particular emphasis on looking for radiologic evidence of otosclerosis, revealed the presence of a focal region of bone demineralization in the region of the fissula ante fenestram. (Figure 1) This finding is consistent with a diagnosis of fenestral otosclerosis.
Otosclerosis ; Hearing Loss, Conductive ; Filipino

BACKGROUND AND OBJECTIVES: To analyse the audiometric profile and the pedigree of a large family with otosclerosis to understand the inheritance pattern and its implication in clinical management of the disease. SUBJECTS AND METHODS: Pedigree analysis was performed on the basis of family history and audiometric tests. Pure tone audiometry, tympanometry, and acoustic reflexes were evaluated for the family members. Audiometric analysis was also carried out for the individuals who have already underwent corrective surgery at the time of study. RESULTS: Out of 112 family members, 17 were affected individuals, and 11 of them were surgically confirmed. Hearing loss (HL) started unilaterally and progressed to bilateral form. Otosclerosis was presented in early 20’s in the first and second generations but it was delayed to mid-late 30’s in the fourth generation. An affected female was diagnosed with otosclerosis during her pregnancy. Though the disease was familial, a mother of four affected offspring in this family did not develop otosclerosis until she died at the age of 84. CONCLUSIONS: The five-generation family, which was analysed in the present study, exhibited autosomal dominant inheritance of otosclerosis with reduced penetrance. Bilateral HL and pregnancy-aggravated otosclerosis were observed in this family. It was found for the first time that the age of onset of the disease delayed in the successive generations. The current study indicated the importance of detailed pedigree analysis for better clinical management of otosclerosis.
Acoustic Impedance Tests ; Age of Onset ; Audiometry ; Family Characteristics ; Female ; Hearing Loss ; Hearing Loss, Conductive ; Humans ; Inheritance Patterns ; Mothers ; Otosclerosis ; Pedigree ; Penetrance ; Pregnancy ; Reflex, Acoustic ; Wills

BACKGROUND AND OBJECTIVES: A free-beam-type CO₂ laser, which use a micromanipulator mounted on a microscope as the delivery system, has the merit of not being affected by hand tremor at the time of shooting. However, this delivery system has several disadvantages, including a restricted operation range and a risk of incorrect focusing. A fiber-type CO₂ laser uses a hand-held delivery system and has the opposite merits and demerits. We compared the results of stapes surgery with free-beam and fiber type delivery systems. SUBJECTS AND METHODS: The study enrolled 36 patients who underwent stapedotomy with free-beam- (n=26) or fiber- (n=10) type CO₂ lasers. The air-bone (AB) gap closure, bone conduction (BC) change, and operating time were evaluated. The AB gap closure was calculated by subtracting the preoperative BC thresholds from the postoperative air conduction thresholds. The BC change was calculated by subtracting the postoperative BC thresholds from the preoperative BC thresholds. RESULTS: The mean operating time was significantly (p=0.035) shorter in the fiber-type group (72.5±8.2 min) than in the free-beam-type group (80.5±11.4 min). The mean AB gap closure did not differ significantly (p=0.297) between the free-beamand fiber-type groups (5.8±10.1 and 1.4±6.8 dB, respectively). The mean BC change did not differ significantly (p=0.873) between the free-beam- and fiber-type groups (2.4±6.9 and 2.8±5.3 dB, respectively). The hearing outcomes did not differ significantly between the two groups. CONCLUSIONS: Operating times were significantly shorter using the fiber-type CO₂ laser, while hearing outcomes did not differ significantly between the two groups.
Bone Conduction ; Hand ; Hearing ; Humans ; Otosclerosis ; Stapes Surgery* ; Stapes* ; Tremor

Conventional impedance audiometry uses only specific tympanometric frequencies (e.g., 226 Hz) and cannot test above 1500 Hz. As many hearing frequencies must be evaluated when diagnosing middle ear pathologies, wideband clicks stimulating tympanometric receptors from 226 to 8000 Hz are commonly delivered. Since wideband tympanometry (WBT) was introduced in 1993, several studies have explored its clinical utility. WBT yields information on absorbances at various frequencies and pressures. Recently, WBT has become increasingly used to diagnose and monitor the hearing of newborns and patients with otosclerosis, ossicular chain disruptions, tympanic perforations, superior semicanal dehiscence syndrome, and middle ear effusions. WBT is also employed for pre/postoperative monitoring using non-pressurized wideband absorbance. Here, we review the concept of WBT, the basic mechanism, and the clinical applications.
Acoustic Impedance Tests* ; Ear, Middle ; Hearing ; Humans ; Infant, Newborn ; Otitis Media with Effusion ; Otosclerosis ; Pathology

OBJECTIVETo analyze the clinical characteristics of unilateral conductive hearing loss with intact tympanic membrane, and summarize the key diagnostic points, differential diagnosis and observe the effects of surgical treatment.

METHODSWe reviewed data from 82 patients with unilateral conductive hearing loss with intact tympanic membranes who accepted the exploratory tympanotomy from April 2011 to September 2013. There were 41 males and 41 females, aged from 7 to 66( averaged 26.5±13.7)years, with a history of one month to 50 years. The history, clinical symptoms, audiological evaluation, high resolution temporal bone CT, the results of surgical exploration and hearing reconstruction were analyzed.

RESULTSThe exploratory tympanotomy revealed 43 cases of congenital middle ear malformations (52.4%), 22 cases of otosclerosis (26.8%), eight cases of congenital cholesteatoma (9.8%), six cases of trauma induced conductive hearing loss (7.3%), three cases of congenital ossicular malformations with congenital cholesteatoma (3.7%). Progressive hearing loss was common in patients with otosclerosis and congenital cholesteatoma, and patients with congenital middle ear malformations described their hearing loss since childhood. High resolution temporal bone CT of congenital middle ear malformation, trauma induced conductive hearing loss, congenital cholesteatoma diagnosis rate was 40.0%, 50.0%, and 83.3% respectively. The preoperative air-conductive threshold of patients with absence of the oval window were increased to (66.9±1.1)dBHL, the preoperative bone-conductive threshold achieved (28.3±10.4)dBHL at 2 000 Hz. While patients with stapes fixation and that with ossicular chain discontinuity were (27.2±9.7)dBHL and (17.8±8.8)dBHL(P=0.000)respectively. Through the tympanic exploration with endaural incision under the microscope, different hearing reconstruction were applied according to different lesions. After the operation, the hearing level of 52 patients with return visit were improved, the mean air-conductive threshold were decreased from (60.0±11.4)dBHL to (32.2±12.1)dBHL(P=0.000); and the mean ABG were decreased from (43.2±12.0)dB to (16.3±9.4)dB(P=0.000).

CONCLUSIONSCongenital middle ear malformations, otosclerosis, congenital cholesteatoma are the most common causes in unilateral conductive hearing loss with an intact tympanic membrane. The diagnosis rate can be improved by analyzing the clinical features. Through exploratory tympanotomy and hearing reconstruction, we can clarify the diagnosis and achieve a satisfying hearing recover.

Adolescent ; Adult ; Aged ; Audiometry ; Child ; Cholesteatoma ; congenital ; pathology ; Diagnosis, Differential ; Ear Ossicles ; pathology ; Ear, Middle ; abnormalities ; Female ; Hearing Loss, Conductive ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Middle Ear Ventilation ; Otosclerosis ; pathology ; Tympanic Membrane ; Young Adult

OBJECTIVE: To investigate the feasibility of the treatment of otosclerosis using laser stapedotomy with mini incision in the external auditory meatus. METHOD: Thirteen patients(15 ears) with otosclerosis evidence on clinic history. They were all operated using the laser assisted stapedotomy by mini incision in external auditory meatus because of the wide straight canal. Laser resection the tendo musculistapedius and anterior and postrior arch, breaking the articulatioincudostapedia, removing the stapes superstructure, making a hole of 6mm diameter in the rear of stapes footplate by laser drilling, implanting the corresponding length Piston artificial ossicle. RESULT: All the surgeries were successful and the operation time was about one hour. There was only one patient manifested vertigo and nausea after the operation. But the symptoms improved three days later after the expectant treatment. All the incisions were healed in the externals. There was significant difference between the preoperative and postoperative PTA. The air conduct improved in every frequent and the bone conduct improved in 1 kHz, 2 kHz and 4 kHz. CONCLUSION Laser assisted stapedotomy by mini incision in the external auditory meatus in patients having wide straight canal with otosclerosis can shorten the operation time, minimize the tissue damage, fasten the healing of the incision and reduce the complications postoperatively. In addition, the mini incision is beauty and easy to nurse.
Ear Canal ; surgery ; Humans ; Lasers ; Otosclerosis ; surgery ; Postoperative Complications ; Postoperative Period ; Prostheses and Implants ; Stapes ; Stapes Surgery ; Treatment Outcome