Objective:To evaluate surgical strategies and clinical outcomes in supra-labyrinthine cholesteatoma management, providing evidence-based guidance for therapeutic decision-making. Methods:Seven patients with supra-labyrinthine cholesteatoma in our hospital from 2021 to 2023 were enrolled in this study. The clinical manifestations, imaging findings, and surgical outcomes of patients were retrospectively analyzed. A systematic literature review focused on surgical anatomy correlations and imaging-based approach selection. Results:All seven cases of supra-labyrinthine cholesteatoma were unilateral. Preoperative otoendoscopy, CT, and intraoperative findings confirmed that they were classified as supral-abyrinthine cholesteatoma according to Sanna's classification. Two cases were operated entirely with otoendoscopy, three cases used a postauricular approach with microscopic assistance, and two cases involved a combined approach with endoscopy and microscopy. Hearing reconstruction with ossicular prosthesis was performed in five cases, while two cases did not undergo hearing reconstruction due to preoperative anacusis confirmed by both subjective and objective hearing tests. In all seven cases, various segments of the facial nerve were exposed during surgery, but postoperative facial nerve function remained intact, hearing was preserved, no cerebrospinal fluid leakage occurred, and no recurrences have been observed to date（as of June 2024）. Conclusion:With the advancement of imaging techniques and microsurgical technology, early diagnosis and surgical methods for supral-abyrinthine cholesteatoma have significantly improved. Compared to traditional approaches, the newer methods reduce unnecessary complications and offer advantages such as minimal surgical trauma, superior hearing preservation rates, and shorter recovery times with better postoperative neural function. This study reviews recent literature on petroclival cholesteatomas, combined with our own cases, to analyze the classification of supral-abyrinthine cholesteatoma and surgical approach selection. The findings aim to optimize treatment strategies and guide appropriate surgical methods, ultimately improving patient prognosis and quality of life.
Humans ; Cholesteatoma/surgery* ; Ear, Inner/surgery* ; Retrospective Studies ; Treatment Outcome

Objective:To describe a novel approach for intractable Meniere's disease exclusively through a transcanal endoscopic ear surgery（TEES）. Methods:This retrospective chart review included patients with intractable Menière's disease who underwent endoscopic transcanal labyrinthectomy in the Department of Otolaryngology Head and Neck Surgery, Guangxi Hospital Division, First Affiliated Hospital, Sun Yat-sen University, between February 2023 and October 2024. The first 70-year-old woman and the other 67-year-old woman, who underwent multiple conservative treatment and chemical labyrinthectomy during outpatient and hospitalization, had frequent vertigo, tinnitus and severe sensorineural deafness. The TEES approach provided a wide exposure of the oval window. The incus and the stapes were removed, expanded the oval window. The perilymph was suctioned, The saccule, utricule, macula utriculi and macula sacculi were removed. The ampulla tissue of the three semicircular canal were destroyed with the right-angle crochet. The oval window was obliterated using the perichondrium of the tragal cartilage and cartilage. Results:Two patients underwent endoscopic transcanal labyrinthectomy, and no intraoperative or postoperative complications were observed. Vertigo was controlled in 2 patients during the follow-up of 6 to 12 months. Two patients complained of total hearing loss after surgery. Conclusion:Even though this study presents a limited number of cases, endoscopic transcanal labyrinthectomy is a promising, safe, and effective procedure in selected cases. Additional studies are needed to determine the risk-benefit profile of this technique.
Humans ; Aged ; Female ; Meniere Disease/surgery* ; Retrospective Studies ; Endoscopy/methods* ; Otologic Surgical Procedures/methods* ; Ear, Inner/surgery* ; Treatment Outcome

Cochlear Implantation ; Ear, Inner/surgery* ; Hearing Loss, Sensorineural ; Humans

Ear, Inner ; physiopathology ; surgery ; Humans ; Vertigo ; surgery

Cerebellopontine Angle ; anatomy & histology ; Ear, Inner ; anatomy & histology ; Humans ; Microsurgery ; Neuroma, Acoustic ; surgery

Cerebellopontine Angle ; anatomy & histology ; Ear, Inner ; anatomy & histology ; Humans ; Microsurgery ; Neuroma, Acoustic ; surgery

OBJECTIVE: To review the classification of cochlear modiolus deficiency and decision on surgical approach for above case,in order to provide mastery for cochlear implant (CI) indication. METHOD: Basing on temporal bone HRCT pre-operation, CI subjects with modiolus deficiency were defined as following groups: (1) deficiency caused by cochlear dysplasia (Mondini malformation); (2) deficiency caused by dysplasia of cochlear and vestibule (Common cavity malformation); (3) deficiency caused by absence of internal acoustic meatus fundus (IP-III malformation). Three types of surgical approach were utilized: type I, electrode array was introduced through facial recess, enlarged the round window, type II, opened the surface of chchlea, electrode array was introduced through facial recess, fenestration on posterior promontory and then inserted around lateral wall of inner-cochlear cavity. type III, electrode array was introduce through fenestration of lateral semicircular canal and then placed close to the bony wall of common cavity. RESULT: One hundred and sixty-six cochlear modiolus deficiency cases were identified into 3 groups as following: 135 Mondini malformation cases into group a, 18 common cavity malformation cases into group b, and 13 IP-III malformation cases into group c. Surgical approach: type I were used in 136 cases (123 Mondini cases and 13 IP-III cases), while approach type II in 12 cases (12 Mondini cases), and approach type III in 18 cases (18 common cavity cases). Income post-operation of CI: For group a (Mondini malformation), post-activation mean hearing threshold in sound field was 65 dB, speech recognition score is 95% (single finals test) and 25% (signal initials test), while it was 80 dB, 60% and 0 for group b (Conmon cavity malformation), and it was 55 dB, 100% and 45% for group c (IP-III malformation). CONCLUSION The income of speech recognition score for cochlear modiolus deficiency was relatively poor, group b was worst and group c was best, while group a moderate.
Cochlea ; abnormalities ; surgery ; Cochlear Implantation ; methods ; Cochlear Implants ; Ear ; Ear, Inner ; abnormalities ; Female ; Humans ; Male ; Postoperative Period ; Round Window, Ear ; surgery ; Semicircular Canals ; surgery ; Speech Perception ; Temporal Bone ; Vestibule, Labyrinth ; abnormalities

OBJECTIVE: To investigate the intra-operative electrical evoked auditory brain stem response (EABR) characteristics and the hearing and speech rehabilitation effects of cochlear implantation (CI) in patients with internal auditory canal stenosis (IACS). METHOD: A retrospective study was performed on 16 patients with IACS (IACS group) matched with 16 implanted without IACS (control group), who received multi- channel CI because of pre-lingual sensorineural hearing loss. The integrity and functional status of the auditory pathway were assessed by EABR, recording waveforms, thresholds and dynamic ranges intra-operation before CI. Interviewed the implanted parents or teachers, asking them to rate the implanted hearing and speech ability according to Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR). Paired T test was performed to compare scores of CAP and SIR between before and 1 year after CI, while Spearman test was performed to compare correlation between EABR grades and post-operative CAP scores. RESULT: Among the IACS group, 2 cases weren't recorded typical EABR waveforms and without auditory response 1 year after a successful CI. The EABR waveforms in the IACS group were poorer than that in the control group, their EABR thresholds higher than the control group, and their EABR dynamic ranges less than the control group. The hearing and speech rehabilitation after CI showed that the results of CAP and SIR values (3.47 +/- 1.09 and 1.62 +/- 0.50) scored significantly lower than the control group (5.06 +/- 0.79 and 2.59 +/- 0.58) (P < 0.05), but significantly increased compared with pre-operation. Intra-operative EABR grades and post-operative CAP scores showed significant correlation (r = 0.78 , P < 0.05). CONCLUSION Intra-operative EABR can accurately monitor the integrity and functional status of the auditory pathway, be of important clinical value in predicting whether patients can acquire auditory responses with the aid of CI. CI can help patients with IACS to improve the ability of hearing and speech with EABR to screen out compatible implanted.
Adolescent ; Auditory Pathways ; Child ; Child, Preschool ; Cochlear Implantation ; Constriction, Pathologic ; Ear, Inner ; surgery ; Evoked Potentials, Auditory, Brain Stem ; Female ; Humans ; Infant ; Labyrinth Diseases ; physiopathology ; surgery ; Male ; Retrospective Studies ; Treatment Outcome ; Young Adult

OBJECTIVE: To investigate the changes and determine the difference of electrode impedance and neural response telemetry ( NRT) value with cochlear implants in children with malformed inner ear and normal inner ear. METHOD: Eighty-eight children with multi-channel cochlear implants participated in this study. Children were divided into malformed inner ear group and normal inner ear group. To test the electrode impedance values and NRT value. RESULT: The changes of electrode impedance showed the similar changing pattern in two groups. There was not different in electrode impedance values between the two groups (P > 0.05). The changes of electrode neural response telemetry values showed the similar changing pattern in two groups. There was significantly different in electrode impedance values between the two groups (P < 0.05). The NRT were going up higher from electrode 22 to electrode 1. CONCLUSION The values vary are different programming sessions and electrodes during the first 3 months of cochlear implant use. To acquire better hearing frequent programming sessions are necessary.
Adolescent ; Case-Control Studies ; Child ; Child, Preschool ; Cochlear Implantation ; Cochlear Implants ; Cochlear Nerve ; Ear, Inner ; abnormalities ; physiopathology ; surgery ; Electric Impedance ; Electrodes ; Female ; Humans ; Infant ; Male

OBJECTIVE: To discuss the surgical approach of cochlear implantation in patients with common cavity. METHOD: Seventeen patients with common cavity underwent cochlear implantations through facial recess approach or transmastoid lateral semicircular canal approach,according to the preoperative imaging and audiological evaluation. RESULT: Common cavity was opened and electrodes were inserted smoothly in all cases. Facial recess approach was used in 3 patients, while transmastoid lateral semicircular canal approach was used in the others. Intraoperative "gush" occurred in 4 cases. None of the cases developed intraoperative or postoperative complications, such as facial paralysis, meningitis and cerebrospinal fluid leakage. All cases had improvements in hearing. Atypical postoperative EABR responses were detected, the average free filed hearing threshold was 65 dB HL, the average speech recognition score of Chinese vowels was 75%, and the average speech recognition score of Chinese consonant was less than 10%. CONCLUSION (1) Selection of surgical approach for patients with common cavity: if the basal turn of cochlear could be distinguished at the posterior tympanum side of common cavity, the facial recess approach was used; if the cochlear,vestibule and the lateral semicircular canal merged to be a spherical cavity, the transmastoid lateral semicircular canal approach was used. (2) Surgical approach of cochlear implantation affected the postoperative outcomes. (3) Outcomes of cochlear implantation in common cavity patients were much poorer than normal cochlear cases.
Adolescent ; Child ; Child, Preschool ; Cochlea ; abnormalities ; Cochlear Implantation ; methods ; Cochlear Implants ; Ear Diseases ; surgery ; Ear, Inner ; abnormalities ; Female ; Humans ; Infant ; Male ; Retrospective Studies ; Semicircular Canals ; abnormalities ; Vestibule, Labyrinth ; abnormalities