Adult-onset congenital cholesteatoma in the hypotympanum initially presenting as Bell's palsy: A case report.

INTRODUCTION: Cholesteatoma is a rare disease characterized by the accumulation of keratinized squamous epithelial cells in the middle ear or mastoid cavity. Vertigo and facial palsy, which are rare complications, may indicate erosion into the semicircular canals or the fallopian canal. PATIENT CONCERNS: A 40-year-old woman presented to our clinic with progressive right-sided hearing loss over 5 years (primary concern). Approximately 10 years ago, the patient had developed acute right-sided facial weakness with no additional symptoms. A neurologist at another hospital had diagnosed her condition as Bell's palsy and treated it accordingly. DIAGNOSIS: Adult-onset congenital cholesteatoma in the hypotympanum. INTERVENTION: Combined endoscopic and microscopic removal of the cholesteatoma. OUTCOMES: Physical examination revealed slight improvement in right-sided peripheral facial palsy. LESSON: Routine eardrum examination is recommended for patients presenting with isolated peripheral facial palsy. If necessary, a patient should be referred to an otologist for further evaluation and treatment.

Surgical Option for External Auditory Canal Cholesteatoma: A Case Report.

External Auditory Canal Cholesteatomas (EACC), is an exceptionally rare condition with a prevalence of only 0.1-0.5% among new patients1. EACC are known to possess bone eroding properties, causing a variety of complications, similar to the better-known attic cholesteatomas. We describe here the novel surgical management of a case of EACC. She is 38-year-old female who presented with otorrhea for 6 months. Clinical examination and radiological investigations suggested the diagnosis of an external auditory canal cholesteatoma. The patient underwent modified radical mastoidectomy with type 1 tympanoplasty with meatoplasty. Post-operatively, the patient showed marked clinical improvement.

Acquired cholesteatoma after cochlear implants: case series and literature review.

PURPOSE: To assess the prevalence and management of acquired cholesteatoma after cochlear implantation in pediatric and adult patients. METHODS: Retrospective case review of pediatric and adult cochlear implants (CI) followed at a tertiary referral center and literature review of acquired cholesteatoma after CI surgery, to identify its prevalence, cause, and treatment. RESULTS: Nine pediatric CIs were diagnosed with cholesteatoma in seven patients after 6.4 ± 4 years from CI surgery, and two adults after 11.3 and 21.7 years from CI surgery. Thirty-four pediatric cases and 26 adult cases are described in the literature. Cholesteatoma has a prevalence of 0.54% in pediatric CIs, and 1.79% in adult CIs (case series and literature). Adult cases were diagnosed significantly later compared to pediatric cases (Mann-Whitney test, p = 0.0460). Three pediatric cholesteatomas were treated with conservative surgery and preservation of the CI; they all developed recurrent disease. The remaining pediatric cases underwent subtotal petrosectomy with simultaneous CI explantation and staged reimplantation. Only one case recurred. The adult cases underwent simultaneous subtotal petrosectomy, explantation, and reimplantation. Similarly, 33.3% of cases treated with conservative/reconstructive surgery in the literature required revision surgery or conversion to subtotal petrosectomy against 6.2% of subtotal petrosectomies in the literature. CONCLUSIONS: Cholesteatoma after CI is a rare and late-onset complication of CIs. It is more prevalent in the adult CI population, although it affects children significantly earlier. The treatment of choice is subtotal petrosectomy and CI explantation with simultaneous or staged reimplantation.

Comparison of temporalis fascia and cartilage graft over-under myringoplasty for repairing large perforations.

OBJECTIVE: The objective of this study was to compare the operation time, graft success, audiometric outcomes and complications of over-under technique using a temporalis fascia (TMF) and cartilage grafts for the repair of large perforations. STUDY DESIGN: Randomized controlled trial. MATERIALS AND METHODS: 80 large perforations >2 quadrants of eardrum were prospectively randomized to undergo TMF over-under technique group (TFON, n = 40) or cartilage-perichondrium over-under technique group (CPON, n = 40). The graft success rate, audiometric outcomes, and complications were compared among two groups at 12 months. RESULTS: The mean operation time was 56.8 ± 4.2 (range:52-71) min in the TFON group and 37.9 ± 2.8 (range: 31-47) min in the CPON group (P < 0.001). The lost follow-up rate was 3 (7.5 %) patients in the TFON group and 2 (5.0 %) patient in the CPON group (P = 0.644). Finally, 37 patients in the TFON group and 38 patients in the CPON group were included in this study. The graft infection rate was 2 (5.4 %) patients in the TFON group and 2 (5.3 %) patient in the CPON group (P = 0.626), all the graft infection resulted in the residual perforation. The remaining residual perforation was 2 (5.4 %) patients in the TFON group and 1 (2.6 %) patient in the CPON group; the re-perforation was 3 (8.1 %) patients in the TFON group and 0 (0.0 %) patient in the CPON group. The graft success rate was 81.1 % (30/37) patients in the TFON group and 92.1 % (35/38) patient in the CPON group. The mean preoperative and 12-month postoperative ABGs were significantly different in any group (P < 0.01). However, there were no significant difference among two groups regardless of pre-or post-ABGs or ABG closure. No lateralization of the graft or blunting was noted in any group. Four (10.8 %)patients developed atelectasis and one (2.7 %) developed the EAC scarring in the TFON group. Graft cholesteatomas was found in 2 (5.4 %) patients in the TFON group and in 5 (13.2 %) patients in the CPON group (P = 0.449). Three (8.1 %) patients had temporary hypogeusia in the TFON group. CONCLUSION: Although temporalis fascia graft over-under technique obtained similar graft success rates and hearing outcomes for large chronic perforations to the cartilage-perichondrium over-under technique, temporalis fascia graft technique prolonged the operation time and increased the re-perforation and graft atelectasis. Nevertheless, the graft cholesteatomas were comparable among two techniques.

Attic reconstruction techniques in cholesteatoma surgery: a comparative analysis of cartilage versus bone pate.

BACKGROUND: Cholesteatoma surgery involves canal wall down (CWD) and canal wall up (CWU) mastoidectomy. CWU is associated with higher cholesteatoma recurrence, often linked to attic retraction pockets. Attic reconstruction with cartilage or bone pate lacks comparative evidence. AIMS/OBJECTIVES: To compare the effectiveness of cartilage and bone pate in attic reconstruction during CWU mastoidectomy for cholesteatoma. MATERIAL AND METHODS: We conducted a retrospective study at King Abdullah University Hospital (KAUH) in Jordan, analyzing surgeries performed from 2011 to 2021. Patients who underwent CWU mastoidectomy with attic reconstruction using tragal cartilage with perichondrium or bone pate were included. RESULTS: Of 48 patients analyzed, 26 had cartilage graft attic reconstruction, and 22 received bone pate. Recurrent cholesteatoma occurred in 19.23% of the cartilage group but none in the bone pate group (p = .001). Ear discharge was observed in 19.23% of the cartilage group and 18.18% of the bone pate group, while tympanic membrane perforations and external auditory canal cholesteatoma were more prevalent in the cartilage group. CONCLUSIONS AND SIGNIFICANCE: Our study indicates that bone pate results in significantly lower cholesteatoma recurrence than cartilage grafting in CWU mastoidectomy attic reconstruction. Bone pate offers stability and favorable long-term outcomes for outer attic wall repair.

Congenital Retrosigmoid Cholesteatoma: Case Series and Literature Review.

BACKGROUND: This study aimed to discuss 3 cases of congenital cholesteatoma located posterior to the sigmoid sinus, with no/minimal involvement of mastoid, and compare them with cases presented in the literature to better define this rare entity. METHODS: Retrospective chart analysis of 3 congenital cholesteatomas located posterior to the sigmoid sinus treated surgically in 2 skull-base centers and literature review. Though congenital cholesteatoma can arise outside the middle ear, only a few cases presenting in the retrosigmoid occipital bone have been described earlier. RESULTS: In all 3 patients, there was a delay in the presentation, as symptoms were nonspecific or lacking, leading in 1 case to severe complications. Computed tomography and magnetic resonance imaging, especially diffusion-weighted imaging scans, allowed accurate diagnosis and surgical planning. Surgery happened to be challenging due to the tight adherence of the cholesteatoma to the thinned dural surface. Complete excision was achieved in all the cases. CONCLUSION: Congenital cholesteatoma located posterior to the sigmoid sinus is a rare entity and is even more exceptional after a critical review of the literature. Complete excision is quintessential to prevent intradural extension or infection. The most important surgical issue is the management of the posterior fossa dura and the sigmoid sinus. We recommend meticulous dissection with slow peeling of the epithelial lining from the dura. Bipolar coagulation of the dura may help in avoiding recidivism. Moreover, cerebrospinal fluid (CSF) leak during dissection has to be avoided as long as possible, because the loss of tension of the already thinned dura makes its peeling particularly difficult.

Diagnosis and management of bilateral lipoma of the middle ear.

INTRODUCTION: Middle ear lipomas are rare, accounting for less than 1% of all middle ear tumours. To our knowledge there have been only 16 reported patients with middle ear lipoma. Only one of these had bilateral congenital middle ear lipomas. The aim of this review is to comprehensive overview the diagnosis and management of congenital lipomas of the middle ear. METHODS: A literature review was performed searching multiple data bases, using the key words "middle ear", AND "lipoma", AND "congenital". A total of 116 articles were identified. One hundred were excluded on various criteria, leaving 16 articles for detailed review. Additionally, we report the second case of bilateral congenital middle ear lipoma. RESULTS: Based on this case report and literature review, the incidence of middle ear lipomas is greater in paediatric patients than in adult patients. Middle ear lipomas are distributed evenly between males and females. Three patients had recognised genetic syndromes, while five had prior ventilation tube placement. Clinically patients present with conductive hearing loss and middle ear effusions. In some cases, presence of a white or yellow mass behind the anterior aspect of the tympanic membrane is recognised. The most common location of the masses is in the anterosuperior quadrant of the middle ear, the site of predilection for small congenital cholesteatomas. An MRI scan is most useful in distinguishing between lipoma and cholesteatoma. All cases reported surgical excision of the middle ear lipoma. There was no evidence of recurrence noted up to 4 years follow up. CONCLUSION: Though rare, congenital lipomas of the middle ear should be a differential diagnosis of a middle ear masses. Early identification can allow surgical lipoma resection with minimal disruption to the ossicular chain. Though there is currently no consensus regarding management, surgical removal and appropriate reconstruction of the ossicular chain if needed, is appropriate.

Analysis of Paparella Type 1 tympanostomy tubes in pediatric patients: A single-center retrospective review.

OBJECTIVE: This study aims to evaluate the demographic characteristics, indications for surgery, clinical follow-up results and complication rates of pediatric patients who have received a Paparella Type 1 tympanostomy tube (TT) insertion. METHODS: Retropective review of 816 ears of 442 pediatric patients who received Paparella type 1 tympanostomy tube insertions was performed. The patients' age, indication for surgery, middle ear effusion, time to extrusion and postoperative complications were analyzed retrospectively. Ears operated for chronic otitis media with effusion (COME) and recurrent acute otitis media (RAOM) were included in the study. Ears that underwent tympanostomy tube insertion for middle ear atelectasis and suppurative complications of acute otitis media were excluded from the study. Ears with middle ear effusion mucoid and serous were included. Ears without middle ear effusion or with purulent effusion were excluded from the study. Patients with a cleft palate, Down syndrome, craniofacial anomalies and those without regular follow-up until their tubes were extruded, were excluded from the study. RESULTS: The mean age of surgery was 5.11 years. 54.3 % of the patients were male and 45.7 % were female. 734 (90 %) tube insertions were performed for patients with COME and 82 (10 %) for those with RAOM. Mucoid middle ear effusion was observed in 86.9 % and serous in 13.1 %. The mean extrusion time of the tubes was 7.16 months. 93.1 % of the tubes were extruded spontaneously within 1 year and 99.9 % within 2 years. Postoperative complications of patients that were included were 8.7 % with otorrhea, 7.7 % premature extrusion, 8.2 % tube occlusion, 0.2 % displacement into the middle ear, 8.2 % tympanic membrane changes (5.4 % sclerosis, 2.3 % retraction and 0.5 % atrophy), 1.2 % permanent perforation, 0.1 % cholesteatoma and 0.1 % retained their tube. Premature extrusion was found to be significantly higher in the RAOM group compared with the COME group (p = 0.042). Tube extrusion time did not affect tympanic membrane changes (p = 0.061). CONCLUSIONS: Complication rates after Paparella Type 1 tube insertion are low. The incidence of complications such as otorrhea and tube occlusion were not significantly different between the indication and middle ear effusion groups. Compared to COME group, premature extrusion were found more frequently in the RAOM group. Complications of displacement into the middle ear, permanent perforation, cholesteatoma and retained tube were much rarer.

Canal wall up versus canal wall down mastoidectomy techniques in the pediatric population with cholesteatoma: A systematic review and meta-analysis of comparative studies.

IMPORTANCE: The optimal surgical management of cholesteatoma remains controversial. Within pediatric otolaryngology, one of the most vital points of contention is the selection of canal wall-up (CWU) versus canal wall-down (CWD) procedures. Pediatric cholesteatoma has high rates of recurrence (16%-54%). In adults, there is evidence that the selection of surgical techniques affects recurrence rates. This has not been shown in children. OBJECTIVES: 1. To systematically review the literature on recurrent and residual cholesteatoma after CWU and CWD in children and perform a meta-analysis of the data. 2. To assess the rates of recurrent and residual cholesteatoma between CWU and CWD techniques in pediatric patients. 3. To assess hearing outcomes by evaluating postoperative differences in the air-bone gap (ABG) between CWU and CWD techniques. DATA SOURCES: A systematic search of PubMed, Embase, Scopus, and Cochrane Collaboration was performed from inception to May 1st, 2020, to identify studies that compared CWU and CWD procedures for acquired cholesteatoma in children. STUDY SELECTION: Search records were screened in duplicate by four reviewers. Inclusion criteria consisted of comparative randomized clinical trials and observational studies assessing outcomes of CWU and CWD techniques in the pediatric population. Studies involving patients with congenital cholesteatoma were excluded. DATA EXTRACTION AND SYNTHESIS: Four reviewers working independently and in duplicate systematically reviewed and extracted study data. Dichotomous variables were analyzed as risk ratios (RR), while continuous variables were compared using weighted mean differences (MD). The risk of bias was assessed using the CLARITY Scale. PRIMARY OUTCOMES AND MEASURES: The outcomes were recurrence, residual disease, air-bone gap (ABG), and air conductive (AC) thresholds. RESULTS: After screening 1036 publications, 17 retrospective cohort studies were selected. 1333 children were included; the overall mean age was ten years (SD 7.9), and the overall mean follow-up time was 5.9 years (SD 6.6). CWU and CWD techniques were performed in 60% (796) and 40% (537) cases. We did not find differences in cholesteatoma recurrence (RR: 1.50, 95% CI 0.94; 2.40; n = 544; I2 0%; Tau [2]: 0.00), or rates of residual cholesteatoma (RR 1.51, 95% CI 0.96; 2.38, n = 506; I2: 0%; Tau [2]: 0.00) in patients who underwent CWU and CWD mastoidectomy. The mean air-bone gap was lower with CWU than CWD (mean difference: 7.60, 95% CI -10.65; -4.54; n = 242; I2: 71%; Tau [2]: 5.98). CONCLUSION: and relevance: We show similar rates of recurrence and residual disease after either CWU or CWD tympanoplasty. Our results challenge the fundamental principle of CWD surgery as a standard technique, as there is no difference in rates of recurrence and residual disease in CWU and CWD. Moreover, audiometric results support CWU with improved hearing outcomes. TRIAL REGISTRATION: PROSPERO identifier: CRD42020184029.

External Ear Disease: Keratinaceous Lesions of the External Auditory Canal.

Keratosis obturans (KO) and external auditory canal cholesteatoma (EACC) are two distinct keratinaceous lesions of the external ear. This article reviews the signs, symptoms, pathophysiology, workup, and treatment of each. Patients with either pathology can often be managed in the clinic with debridement; however, EACC is more likely to involve osteonecrosis and require more extensive operative management if disease is not confined to the canal on account of the bony erosion characteristic of cholesteatoma. If required for extensive disease, surgical approaches to both pathologies are similar.

The role of planned two-stage surgery in the management of congenital cholesteatoma.

OBJECTIVE: The purpose of this study is to verify the role of "planned two-stage surgery" in the management of advanced congenital cholesteatoma regarding disease recurrence rates, complications and the need for salvage surgery. METHOD: Retrospective review of all congenital cholesteatoma under the age of 18 years underwent surgery from October 2007 to December 2021 in a single tertiary referral center. Patients with Potsic stage I/II who had closed-type congenital cholesteatoma received one-stage surgery. Advanced cases or those with open-type infiltrative congenital cholesteatomas underwent planned two-stage surgery. The second stage of surgery was performed 6-10 months after the first stage of surgery. Ossiculoplasty would be performed in the second operation if a significant air-bone gap was detected in the preoperative pure-tone audiometry test. RESULTS: Twenty-four patients were included in the series. Six patients received one-stage surgery and no recurrence was noted in this group. The remaining 18 underwent planned two-stage surgery. Residual lesions found in the second operative phase were observed in 39% of patients who received planned two-stage surgery. Except for one patient whose ossicular replacement prosthesis protruded and two patients who had perforated tympanic membranes, none of the 24 patients required salvage surgery during follow-up (mean, 77 months after surgery), and no major complications occurred. CONCLUSIONS: Planned two-stage surgery for advanced-stage or open infiltrative congenital cholesteatoma could timely detect residual lesions to avoid extensive surgery and reduce complications.

[Partial sequestration of the labyrinth in a child with chronic suppurative otitis media with cholesteatoma]. / Chastichnaya sekvestratsiya labirinta u rebenka s khronicheskim gnoinym srednim otitom s kholesteatomoi.

A clinical case of a 16-year-old child with chronic suppurative otitis media with cholesteatoma complicated by partial labyrinth sequestration is presented. The case describes is a rare disorder. Computed tomography of temporal bones consistent with intraoperative data was decisive in establishing the diagnosis and the surgery extent.

[Anatomical factors of temporal bone residual cholesteatoma: our experience]. / Osobennosti stroeniya visochnoi kosti kak faktor retsidiva kholesteatomy: nash opyt.

The article describes clinical cases of invasive cholesteatoma of the temporal bone pyramid, leading to a massive destruction of the surrounding structures. Paying attention to the importance of knowing the microsurgical anatomy of critical structures of temporal bone (the anterior bony plate (cog), the tensor tympani fold and the tendon, the tympanic orifice of the eustachian tube, the pretympanic recess, tympanic sinuses) bearing a significance and helping to improve the removal of the invasive cholesteatoma.

Time Analysis of Recidivism in Acquired Cholesteatoma Using the Kaplan-Meier Method.

OBJECTIVE: To analyze the time trends of recidivism of acquired cholesteatoma using the Kaplan-Meier method. STUDY DESIGN: We conducted a retrospective, observational study of 256 patients having their first cholesteatoma surgery. The cumulative recidivism-free rate was calculated using Kaplan-Meier survival analysis related to the follow-up period, pathophysiology, the extent of the disease, and recidivism pathologies. RESULTS: Pars flacida cholesteatoma with tympanic cavity progression had a high likelihood of recurrence disease. Pars tensa cholesteatoma led to more recurrence of the disease than the residual disease. In both pars flacida and pars tensa cholesteatoma, the incidence of disease recurrence increased even 3 years after surgery. On the contrary, the incidence of residual disease peaked within 3 years after surgery, and thereafter, the incidence of residual disease tended to be small. In particular, pars flacida cholesteatoma extending into the mastoid cavity or tympanic cavity tended to recur up to 5 years postoperatively. CONCLUSIONS: We calculated the cumulative recidivism-free rates of 256 patients with cholesteatoma using Kaplan-Meier survival analysis. These results can lead to better estimates of the length of the follow-up period. LEVEL OF EVIDENCE: Level IV evidence from case-control studies.

Endaural microscopic approach versus endoscopic transcanal approach in treatment of attic cholesteatomas.

PURPOSE: Compare the audiological results and postoperative outcomes of the endoscopic approach versus the endaural microscopic approach for treatment of attic cholesteatomas, using a randomized prospective model. MATERIALS AND METHODS: Eighty patients were consecutively enrolled in the study and randomized into two groups of treatment of 40 patients: Group A -tympanoplasty with a microscopic endaural approach; Group B -tympanoplasty with an exclusive trans-meatal endoscopic approach. Preoperative, intraoperative and postoperative outcomes were evaluated. Hearing was assessed preoperatively and at 1 month, 3 months and 6 months after surgery in both groups. RESULTS: There were no differences in the parameters analyzed (CT findings, patient age, disease duration, intraoperative cholesteatoma characteristics,) between the group A and B patients. No statistical difference between the two groups regarding hearing improvement, abnormal taste sensation, dizziness, post-operative pain and healing times emerged. Graft success rate was 94.5 % and 92.1 % for MES and ESS respectively. CONCLUSION: Both microscopic and exclusively endoscopic endaural approaches offer similar and excellent results in the surgical treatment of attic cholesteatomas.

Intratympanic membrane cholesteatoma after traumatic tympanic membrane perforation: a case report.

BACKGROUND: Intratympanic membrane cholesteatoma presents as an asymptomatic, white, round mass on the tympanic membrane, and is usually detected incidentally in children. CASE PRESENTATION: A 12-year-old Korean boy visited our otorhinolaryngology clinic for a whitish mass on the right tympanic membrane. He had a history of traumatic tympanic membrane perforation in the right ear that had occurred 1 year prior, which had healed well with a paper patch placement. The mass was completely removed under local anesthesia during surgery with a microscope. The mass was on the outer epithelial layer of the right tympanic membrane and did not invade the middle fibrous and inner mucosal layers. Cholesteatoma was diagnosed on the basis of histopathology. CONCLUSION: Intratympanic membrane cholesteatoma may not induce symptoms or invade the middle ear because it can grow outwards into the external auditory canal. However, intratympanic membrane cholesteatoma can grow over time, and then after growth, it can compress the tympanic membrane and advance into the middle ear, which can cause symptoms such as hearing loss. Intratympanic membrane cholesteatoma in children should be carefully evaluated and followed, and surgical removal should be considered, even for asymptomatic cases, to minimize potential damage and hearing loss.

[Diagnosis and treatment of congenital temporal bone cholesteatoma].

Congenital temporal bone cholesteatoma is a rare lesion in otolaryngology.The disease is locally invasive and may lead to significant complications,including hearing loss（conductive or sensorineural）, temporal bone destruction and intracranial invasion. This article reviews the characteristic symptoms of congenital temporal bone cholesteatoma, testing and imaging of the disease, stage and the current treatment options in order to promote awareness to this rare disease entity and perform early surgical treatment, effectively avoid the destruction of the temporal bone and its surrounding structures, thereby reducing the occurrence of complications. By improving the understanding of the disease and performing early surgical treatment, the destruction of the temporal bone and its surrounding structures can be effectively avoided, thereby reducing the occurrence of complications.

Epigenetic Regulation as a New Therapeutic Target for Middle Ear Cholesteatoma.

HYPOTHESIS: To evaluate the effectiveness of the menin-MLL inhibitor, MI503, as a conservative treatment of middle ear cholesteatoma (cholesteatoma) in a mouse model and to confirm its safety profile regarding auditory function in vivo. BACKGROUND: Cholesteatoma is a mass formed by the keratinizing squamous epithelium in the tympanic cavity and/or mastoid and subepithelial connective tissue and by the progressive accumulation of keratin debris with/without a surrounding inflammatory reaction. Although the main treatment is surgical therapy, the techniques to prevent recurrence remain a critical area of research. Recently, the use of MI503 in experiments resulted in the inhibition of the growth of cholesteatoma in vivo under histone modification. METHODS: After cholesteatoma was induced in ICR mice (n = 7) by keratinocyte growth factor expression vector transfection, MI503 (50 µM) or phosphate-buffered saline was topically injected for 14 days. The effects of MI503 against cholesteatoma were analyzed by micro-computed tomography images. For the in vivo ototoxicity study, a single intratympanic injection of MI503 (50 or 500 µM) or phosphate-buffered saline (n = 4 each) was done in the ICR mice. An auditory brainstem response was performed at days 0, 1, and 14. For morphological analysis, immunostaining for Phalloidin/F-actin and Myo7a was performed. RESULTS: MI503 reduced keratinocyte growth factor-induced cholesteatoma in vivo (4 of 4 [100%]). No difference was found in the mean variation of the average of the auditory brainstem response thresholds between the three groups in the in vivo ototoxicity study, thus confirming its safety profile regarding auditory function. MI503 does not demonstrate any deleterious effects on murine hair cells when assessed by immunostaining. CONCLUSION: These findings demonstrate an encouraging safety profile for the use of menin-MLL inhibitor for the conservative treatment of cholesteatoma.

Taste Alteration in External Auditory Canal Cholesteatoma: Indicator of Impending N VII Affection.

INTRODUCTION: External auditory canal cholesteatoma (EACC) is a rare disease, with an estimated incidence of approximately 1:1,000 adult and 1.6:1,000 pediatric otologic patients. Systematic studies of chronic ear disease and taste alteration prior to surgery are rare; in fact, there are no such studies for EACCs. Therefore, we describe chorda tympani nerve (CTN) dysfunction and the related clinical consequences in EACC patients. METHODS/STUDY DESIGN: Between 1992 and 2021, we retrospectively analyzed the symptoms, signs, and radiological and intraoperative descriptions of CTN involvement in 73 patients. Liquid taste tests and, since 2009, Taste StripsTM as well as an olfactory screening test (Smell DiskettesTM) have been performed for all symptomatic patients and, when feasible, all other EACC patients. RESULTS: Ten of 73 patients complained subjectively of dysfunction, and 8 showed abnormal taste test results. Four patients complained of olfactory dysfunction (3 cases with pathological taste tests). Gustatory dysfunction was most frequent in radiogenic EACC cases (n = 4), followed by postoperative EACC (n = 3). Two postoperative patients were asymptomatic despite abnormal test results. Rarely, patients with idiopathic (n = 2) and posttraumatic (n = 1) EACC showed acute taste dysfunction that was confirmed in each with abnormal test results. DISCUSSION/CONCLUSION: CTN dysfunction often developed asymptomatically in chronic ears, except for idiopathic and posttraumatic EACCs under previous healthy middle ear conditions. Taste disturbance is not a cardinal symptom of EACC, but objective testing suggests that up to one out of 10 EACC patients with advanced disease may experience regional gustatory dysfunction prior to surgery. Especially in context of a new and acute presentation, regional taste dysfunction may alert the clinician of potential progressive EACC invasion and danger to the facial nerve.