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

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

Objective: To explore the clinical characteristics, intervention and treatment of tympanic osteoma at different locations. Methods: The medical history, audiological and imaging examination, operation and follow-up results of two patients with tympanic osteoma at different sites were reviewed and summarized. Furthermore, the clinical characteristics and interventions of 36 patients reported in literatures with tympanic osteomas were also summarized and analyzed. Results: Osteoma of the two patients collected in this study located at promontory and incus respectively;both of them presented with intact tympanum and conductive deafness, without obvious etiology or predisposing factor. Both of them underwent surgeries and the hearing improved significantly. For patient one, the ossicular chain was intact and restored to activity after removed the osteoma. For patient two, an artificial ossicle was implanted after removed the osteoma and incus. In the 36 patients reported in literatures, the average age was 26.5 years, and 39.47% of them located at promontory; in addition, the main symptoms of them were progressive hearing loss, tinnitus and ear stuffy. Conclusions: Patients with tympanic osteoma are characterized by conduction deafness with intact tympanic membrane, and the most common lesion is promontory. Hearing can be restored by excision of the osteoma and maintenance or reconstruction of the ossicle chain.
Adult ; Ear Ossicles/surgery* ; Ear, Middle/surgery* ; Hearing Loss, Conductive/surgery* ; Humans ; Osteoma/surgery* ; Tympanic Membrane

Objective: To investigate the feasibility and safety of auricle reconstruction combined with Bonebridge implantation for bilateral aural atresia patients. Methods: A retrospective analysis was conducted for 36 cases(72 ears) who underwent Bonebridge implantation combined with bilateral auricle reconstruction from February 1, 2017 to January 15, 2020. All cases were bilateral congenital aural atresia and underwent Nagata auricle reconstruction for both sides simultaneously. Bonebridge implantations were performed during the second stage of auricle reconstruction. Results: All 36 patients healed well and had no surgical complications when discharged. The preoperative average bone conduction threshold of the patients was(8.5±5.8) dB HL and postoperative bone conduction threshold was (8.4±5.2) dB HL. There was no significant change after the implantation (P=0.724). The preoperative average air conduction threshold of was(64.9±7.4)dB HL and postoperative air conduction threshold was (24.0±5.3) dB HL, which had a significant change after the implantation (P<0.001). The hearing threshold with Bonebridge significantly decreased by 40.9 dB HL compared with the preoperative air conduction threshold(P<0.001). The speech recognition rate of monosyllable words, disyllabic words and short sentences in quiet environment increased by 62.5%, 63.5% and 72.2% respectively. The differences were statistically significant (P<0.001). The speech recognition rate of monosyllabic words, disyllabic words and short sentences in noise environment were significantly increased by 55.9%, 58.9% and 69.9% respectively (P<0.001). After a follow-up of 18.3 months in average, the hearing results were stable and the aesthetic outcomes were satisfied. One patient had implant rupture and healed after revision surgery. Conclusions: With an integrated surgical procedure, patients with bilateral congenital aural atresia can complete bilateral auricle reconstruction and hearing implantation within six months. This integrated surgical procedure is safe and efficient, with a stable hearing improvement and good appearance.
Bone Conduction ; Ear, External ; Hearing Aids ; Hearing Loss, Conductive ; Humans ; Retrospective Studies ; Treatment Outcome

Objectives: To evaluate the therapeutic effects of unilateral/bilateral bone conduction hearing rehabilitation in patients with bilateral microtia accompanied with severe conductive hearing loss following staged auricle reconstruction and bonebridge implantation. Methods: Thirty-two patients, including 20 males and 12 females, with an average age of 11.8, who received surgical treatment in Peking Union Medical College Hospital (PUMCH) from March, 2016 to January, 2020 with bilateral microtia-atresia were included. Hearing thresholds, speech perception and high-resolution CT of the temporal bone were evaluated prior to surgery and individualized surgery plans (staged auricle reconstruction and bonebridge implantation) were made. Hearing thresholds and speech perception in quiet and noise (SNR = 5 dB) using unilateral Bonebridge were tested two weeks after the implantation surgery when the Bonebridge was activated and at 3^th, 6^th, 12^th month after activation. Hearing thresholds and speech perception were also tested at least three months after the activation of the Bonebridge under three conditions: unaided, unilateral Bonebridge, and bilateral bone conduction hearing devices (Bonebridge plus contralateral ADHEAR). The international hearing aid assessment questionnaire (IOI-HA) and Glasgow children's benefit questionnaire were used to evaluate the subjective benefits of the patients. SPSS 21.0 software was used for statistical analysis. Results: Among these 32 patients, nine were conducted Bonebridge implantation surgery before auricle reconstruction, six were simultaneously with auricle reconstruction and 17 were implanted after auricle reconstruction surgery. Compared with unaided, the mean hearing thresholds (0.5, 1, 2, and 4 kHz) and speech perception following unilateral BCHD and bilateral BCHD attachment were improved significantly (P<0.05 each). The speech perceptin in noise of bilateral BCHD was better than unilateral (P<0.05 each). The modified questionnaire revealed high levels of patient satisfaction following use of both unilateral and bilateral devices. Conclusions: Individulized surgical procedures involving auricle reconstruction and Bonebridge implantation are safe and effective for patients with bilateral microtia-atresia, solving both appearance and hearing problems. Speech perception in noise is better following bilateral BCHD than unilateral BCHD attachment.
Bone Conduction ; Child ; Congenital Microtia/surgery* ; Ear, External ; Female ; Hearing Aids ; Hearing Loss, Conductive/surgery* ; Humans ; Male ; Speech Perception

Objective: To conclude the clinical features and the postoperative efficacy of congenital middle ear malformation treated with Malleostapedotomy (MS), and to explore the security and effectiveness of MS surgery. Methods: The clinical data of 17 patients (18 ears) with congenital middle ear malformation undergoing MS procedure were analyzed. There were 10 males (11 ears) and 7 females (7 ears), aged from 7 to 48 years. The imaging examination, pure-tone audiometry, intraoperative findings and postoperative hearing improvement of these patients were analyzed and summarized, and software SPSS23.0 was used for statistical analysis. Rusults All the 17 patients (18 ears) presented with hearing loss since childhood on the affected sides. Preoperative high resolution CT (HRCT) of the temporal bone revealed definite malformations in 9 ears (6 ears with incus long process dysplasia and 3 ears with anterior and posterior crus dysplasia). Before surgery, the mean bone conductive hearing threshold at 500, 1 000, 2 000 and 4 000 Hz was (15.6±10.2) dB HL, the mean air conductive hearing threshold was (60.6±9.7) dB HL, and the mean air-bone gap was (45.0±8.9) dB. During the surgery, all 18 ears were found to be accompanied by absence or hypoplasia of incus long process. 12 ears had stapes fixation, 6 ears had oval window atresia. All patients were treated with MS procedure by using Piston. The patients were followed up for 3 months to 1 year. The mean bone conductive hearing threshold was (14.7±8.8) dB HL. The mean air conductive hearing threshold was (37.7±11.6) dB HL, and the mean air-bone gap was (23.0±8.0) dB. There were statistically significant differences in the mean air conductive hearing threshold and mean air-bone gap before and after surgery (P<0.05). While there were no statistically significant differences in the mean bone conductive hearing threshold before and after surgery (P=0.550). Conclusions: MS procedure is safe and reliable in patients with congenital middle ear malformation of incus long process dysplasia, stapes fixation or oval window atresia. HRCT is useful in evaluating the major deformity of ossicular chain and facial nerve deformity. However, it is not enough to evaluate the joint of incus-stapes and oval window atresia. MS surgery in middle ear malformation requires advanced surgical experience and skills. The hearing improvement can be significant, even though some air-bone gap after surgery exist.
Child ; Ear Ossicles ; Ear, Middle/surgery* ; Female ; Hearing Loss, Conductive/surgery* ; Humans ; Male ; Retrospective Studies ; Stapes ; Treatment Outcome

Objective: To evaluate the auditory efficacy and subjective satisfaction of adhesive bone conduction hearing aid in children with unilateral congenital aural atresia (UCAA). Methods: Ten subjects (5 males and 5 females) diagnosed with UCAA with an average age of 8.3 years old (ranged from 5 to 15) were included in Beijing Tongren Hospital, Capital Medical University from January to August 2019. The free sound field hearing threshold, word recognition score in quiet, speech reception threshold in noise and sound localization ability (results were measured by RMS error) tests were performed in unaided and aided situation, respectively. Subjective satisfaction questionnaires were also distributed to subjects. Paired t test and Wilcoxon signed rank test were used as statistical analysis methods. Results: The average hearing threshold in aided condition was improved by (21.9±4.4) dB (t=15.8，P<0.05). Speech recognition abilities were generally improved both under quiet and noise (P<0.05);however, when the binaural summation, squelch and head shadow effects were analyzed respectively, the binaural squelch effect was not statistically improved (P>0.05), while the other effects were improved in aided condition (P<0.05). In sound localization test, there was no significant difference of the RMS error value between the unaided and aided situation (P>0.05). The subjects got high satisfaction rates in three subjective questionnaires. Conclusion: The adhesive bone conduction hearing aid can provide significant audiological benefit for children with UCAA as well as raising the quality of their life.
Adhesives ; Adolescent ; Bone Conduction ; Child ; Child, Preschool ; Female ; Hearing Aids ; Hearing Loss, Conductive ; Humans ; Male ; Speech Perception ; Treatment Outcome

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

Primary tumors arising from the external auditory canal (EAC) are rare. We describe two cases of mass lesions within the EAC causing slowly progressive hearing loss without otorrhea or otalgia. Otoendoscopic examination demonstrated total obstruction of the EAC, and pure tone audiometry revealed conductive hearing loss. Based on the findings of the histopathologic examination, one patient was diagnosed with venous hemangioma that was treated using surgical resection, and the other patient was diagnosed with diffuse large B-cell lymphoma (DLBCL) that was treated using external-beam radiation therapy. Although primary tumors in the EAC are rare, both benign tumors such as venous hemangiomas and malignant lesions such as DLBCL should be considered as possible differential diagnoses of mass lesions in the EAC.
Audiometry ; Diagnosis, Differential ; Ear Canal ; Earache ; Hearing Loss ; Hearing Loss, Conductive ; Hemangioma ; Humans ; Lymphoma, B-Cell

Middle ear adenoma is a very rare disease which is benign and originates from the middle ear mucosa. Patients of middle ear adenoma usually come to the clinic for unilateral hearing loss or tinnitus, but rarely for accompanied facial palsy. It is non-gender specific and occurs over a wide range of ages. The recurrence rate is known to be very low, but few authors argue that neuroendocrine adenoma should be considered as a low grade carcinoma due to some cases of recurrence. A 18 years-old male who had a left side facial palsy about 3 years ago but has currently improved as compared with the initial onset, visited our clinic for the left side hearing loss. Pure tone audiogram showed about 30 dB of conductive hearing loss and a pinkish polypoid mass involving the left tympanic membrane. We removed a tumor via transmastoid approach. The final diagnosis was middle ear adenoma with neuroendocrine differentiation. Neither signs of complication nor recurrence were observed after six months of the surgery.
Adenoma ; Diagnosis ; Ear, Middle ; Facial Paralysis ; Hearing Loss ; Hearing Loss, Conductive ; Hearing Loss, Unilateral ; Hearing ; Humans ; Male ; Mucous Membrane ; Rare Diseases ; Recurrence ; Tinnitus ; Tympanic Membrane