Optimizing Management of Otophyma: A Case Series Highlighting the Role of Surgical and Retinoid Therapy

Abstract

Otophyma is a rare condition that can result in conductive hearing loss. Current otophyma literature does not examine validated treatment outcomes for patients. Utilizing a medical and surgical approach to maintain a patent canal can lead to significant objective improvements. The aim of this case series is to describe a combined successful approach in 3 cases from an academic, multidisciplinary center. The main outcomes analyzed were pre and post air-bone gap audiogram analysis and disimpaction frequency. The results showed that post-management, patient 1 had substantial improvement in hearing, recovering 49 dB in his right ear and 25 dB in his left ear, demonstrating near complete air-bone gap closure. Patient 2 showed a similar dermatologic and functional improvement, although objective audiometric assessment related to otophyma could not be performed due to coexisting chronic otitis media and cholesteatoma. Patient 3, in the 12 months prior to comanagement, had 8 bilateral disimpactions, and following comanagement had 2 disimpactions in 23 months. All 3 patients were pleased with the resultant functional and physical appearance following comanagement. By presenting this approach and objective measures of treatment, we hope to improve future clinical decision-making in a rare condition.

Introduction

Otophyma is a poorly referenced condition, associated with other phymatous changes of rosacea (see Table 1 for literature review). We present 3 otophyma cases with associated hearing abnormalities, rosacea, and rhinophyma, to describe a combined surgical and medical management approach.

Table 1.

Review of Current Literature Regarding Otophyma.^a

Study	Methods	Interventions	Outcomes
Carlson et al. Otophyma: a case report and review of the literature of lymphedema (elephantiasis) of the ear	Single case report of male in his 50s	Corticosteroids and minocycline therapy regimen	Resulted in partial reduction of ear enlargement
Gupta et al. Otophyma: a rare and frequently misdiagnosed entity	Single case report of female in 50s	Was advised biopsy but preferred and received complete excision of swelling	Not discussed
Alcántara-Reifs et al. Otophyma: a rare variant of phymatous rosacea	Single case report of 49-year-old male	Initially treated with metronidazole 0.75% gel BID and doxycycline 100 mg/d	At 4 months of treatment, the patient showed improvement with a reduction of the skin thickness, ear size, edema, and erythema. Thus, doxycycline was tapered to a maintenance dose and metronidazole therapy was continued.
Sharma et al. Treatment of otophyma: case report and review of the literature	Single case report of 41-year-old female	Years of unsuccessful management with topical emollients, steroids, regular ear toileting Now, treated with excision of phymatous tissue and full thickness grafting	Preoperative audiometry demonstrated conductive deafness of 15-30 dB in the right ear and 10-15 dB in the left ear Postoperative audiometry revealed thresholds within the normal range of hearing bilaterally
Azizzadeh-Roodpishi et al. Otophyma in a woman: a rare and neglected clinicopathological entity.	Single case report of 48-year-old female	Treated with various systemic antibiotics and topical corticosteroids Treated with isotretinoin 20 mg/d for 4 months Referred to otolaryngologist for surgical intervention	No improvement with medical management (antibiotics, steroids, isotretinoin) Surgical outcome not discussed
Daniels et al. Otophyma: a case report	Single case report of 46-year-old male	Patient underwent excision of swelling using a potassium titanyl phosphate laser	Achieved good cosmetic appearance with no signs of reoccurrence
Bednarek et al. Otophyma in Morbihan disease	Single case report of 62-year-old male	Metronidazole cream, brimonidine gel, and oral antibiotics had been attempted Patient was started on isotretinoin 40 mg/d with plans to continue for 24 months	Minimal improvement with medical management Results of isotretinoin course not discussed
Kahn et al. Excisional debulking and electrosurgery of otophyma and rhinophyma	Single case report of 65-year-old male	Clindamycin 1% topical and oral antibiotics Underwent excisional debulking with electrosurgery	No improvement with medical management 10-week follow-up showed overall improved quality of life
Shuster et al. Otophyma: a rare benign clinical entity mimicking leprosy	Single case report of 62-year-old male	Hydrocortisone cream Patient referred to plastic surgery for staged ear reconstruction	No improvement with cream Surgical outcomes not discussed
Ezra et al. Gnatophyma and otophyma	Single case report of 73-year-old male	17-year progressive history	Patient’s results not described
Ekmekci et al. A case of otophyma	Single case of 31-year-old male	Oral ornidazole 500 mg daily was administered for 3 weeks Then oral isotretinoin 50 mg daily was given for 3 months Surgical management was declined by patient	Mild improvement was noted with ornidazole After 3 months of isotretinoin, there was fading erythema, regression of follicular plugs, and some diminution of bulk in the ear observed
Wollina et al. Otophyma, rhinophyma and telangiectatic rosacea—a rare combination in a female patient	Single case report of 50-year-old female	Minocycline 50 mg BID for 6 weeks combined with topical metronidazole ointment for the face and topical fusidic acid for outer ear canals	Stepwise improvement in inflammation and swelling. Treatment was well tolerated.

^a Search Strategy: A literature search was undertaken using PedMed, Embase, Scopus, and Web of Science using the following search criteria: otophyma OR ([ear OR otic] AND [rosacea OR phyma OR phymas OR phymatous]). The above searches were performed with the following limitations: English language, humans, past 20 years.

Case Reports

A male in his 40s presented to dermatology with a 20+ year history of bilateral ear phymatous changes complicated by progressive hearing loss (Figure 1). After multiple biopsies and diagnoses, the patient underwent bilateral canaloplasty and meatoplasty leading to significant improvement and was started on isotretinoin for long-term management (see Table 2).

Table 2.

Presenting 3 Case Reports Examining Outcomes of Comanagement.

Patient no/sex/age at presentation/comorbid conditions	1/M/40s/HTN, diabetes, asthma, hyperlipidemia, gout	2/M/50s/HTN, CAD, OSA, hypothyroidism, asthma	3/M/60s/posterior vitreous detachment
Prior diagnoses given	Bilateral chronic otitis externa, autoeczematous dermatitis, relapsing polychondritis, granulomatous rosacea, severe bilateral otophyma	Bilateral chronic diffuse otitis externa, rosacea, chronic mastoiditis	Bilateral keratosis obturans of external ear canal
Preexisting rosacea	Yes	Yes	Yes
Rhinophyma	Yes	Yes	Yes
Presenting signs & symptoms	Pruritus, drainage, hearing loss, minimal erythema, edema	Edematous ears, hearing loss, tinnitus, ear drainage	Tinnitus, hearing fullness/plugging, hearing loss, pruritis
Pertinent PE findings	Diffusely thickened, indurated large, bilateral obstructing fungating masses in conchal bowls	External ears described as cauliflower or chondritic in appearance, very edematous with tympanic membrane not fully visible	Bilateral eczematous external canal, bilateral thick keratosis plug obstructing tympanic membrane
Main biopsy results	Punch Biopsy, right ear: Perivascular and periadnexal dermatitis and associated dermal fibrosis—near one edge of the specimen is a small focus of granulomatous inflammation identified in the fibrotic dermis on the deepest levels of sectioning obtained Punch Biopsy, right ear: Superficial and deep perivascular dermatitis with associated degeneration of cartilage and perichondral fibrosis. Comment: These findings are most compatible with relapsing polychondritis. Excisional biopsy, left ear canal: Chronic inflammation, scar, and degenerative changes of cartilage Excisional biopsy, right conchal bowl: Denuded epidermis with underlying chronic dermal inflammation and fibrosis, benign.	Punch biopsy, right ear: Hyperkeratosis including “shouldering” parakeratosis overlying an epidermis showing moderate acanthosis and mild to moderate spongiosis. Exocytosis of small, cytologically bland, mature appearing lymphocytes is observed. The dermis contains a superficial, perivascular, chronic inflammatory cell infiltrate composed of mostly bland lymphocytes. A few plasma cells are noted.	No biopsies to review
Previously unsuccessful therapies	Kenalog injections, #2 accessory tragus removal Prescribed amoxicillin/clavulanate course Dapsone 50 mg prescribed after biopsy raised suspicion for relapsing polychondritis (dapsone was chosen based on patient’s comorbidities and was titrated up to 200 mg over the course of a year) CO2 laser + serial ILK 40 injections Doxycycline 100 mg prescribed due to high suspicion of granulomatous rosacea Metronidazole prescribed for treatment of otophyma while awaiting surgery as well as azithromycin (later was changed to minocycline) and tazarotene	Had 4 unspecified ear surgeries in 1982, 1991, 1995, 2015 prior to presentation First tried 1% adapalene gel (due to insurance coverage) for 3 months	Numerous bilateral cerumen impaction removals Clotrimazole 1% solution for suspected fungal infection
Successful therapy	Left canaloplasty, left meatoplasty for otophyma. A few months later, patient had right canaloplasty, right meatoplasty. Isotretinoin (1 tablet) started after surgery Post canaloplasty, patient objectively and subjectively saw improvement. Successful isotretinoin response (improved hearing, ears less edematous).	Isotretinoin with transition to acitretin for long-term use Left canal wall down mastoidectomy without ossicular chain reconstruction (OCR) plus meatoplasty Postsurgery, patient reported hearing was improved with minimal ear drainage Patient reported improvement in bilateral ears almost a year following the combined surgical and medical approach.	Patient saw improvement with keratosis and ear fullness with acitretin and adapalene 0.3% gel at 5 months. Symptoms worsened after medication was discontinued Medication was resumed, showing an exceptional response to the acitretin/adapalene combination therapy Transitioned to adapalene gel for long-term management
Outcomes of therapy premanagement and post-management	Initially, the air-bone gap in the left ear was 29 dB, which improved to 4 dB post-management. Initially, the air-bone gap in the right ear was 58 dB, which improved to 9 dB post-management. From a dermatologic perspective, patient was very pleased with results in appearance of ears.	Although the classic skin findings were present in this case, the patient had previous ear surgeries, which made the goals of management different. The goal was to provide a clean, dry, and safe ear for the patient, which was accomplished. The goal of improving hearing loss was not feasible given his underlying mixed hearing loss and concurrent middle ear disease. From a dermatologic perspective, the patient reported improvement with his ear appearance.	Patient did not have pre and post management audiograms to review. However, patient has been able to space his previously frequent ear disimpactions, notes decrease in ear edema, and subjective hearing improvement.

Abbreviations: CAD, coronary artery disease; HTN: hypertension; OSA, obstructive sleep apnea; PE, physical exam.

Figure 1.

Left: preoperative photo, post lidocaine injection of patient one’s left ear. Right: postoperative follow-up photo of patient one’s left ear.

A male in his 50s presented to otolaryngology with bilateral hearing loss. He was referred to dermatology for isotretinoin, which reduced his ear edema, after which he underwent left mastoidectomy for associated chronic otitis media and cholesteatoma. Due to concerns with recurrence, he was transitioned to acitretin for long-term management.

A male in his 60s presented to otolaryngology with bilateral enlarged auricles and canal stenosis, which required monthly cerumen disimpactions. After 6 months of acitretin and adapalene 0.3% gel therapy, he noted subjective hearing improvement, fewer disimpactions, and was transitioned to adapalene gel for long-term management.

Discussion

Otophyma describes unilateral or bilateral ear enlargement characterized by fibrosis, sebaceous hyperplasia, and edema. ¹ These phymatous changes occur in the outer third of the external auditory canal (EAC) where the sebaceous glands reside. While otophyma is poorly cited, there are several cases of bilateral ear enlargement with biopsies supporting the diagnosis of otophyma. ^{1 -12} These patients were treated with a variety of medical therapies including metronidazole, doxycycline, dapsone, and prednisone without objective verification of improvement. Most literature emphasizes including otophyma in the differential for ear enlargement. The differential is broad encompassing other edematous conditions including relapsing polychondritis, chronic dermatitis, and chronic otitis externa. ¹² Consequently, many patients go through an extensive workup receiving antibiotic and steroid treatments before the final otophyma diagnosis is made. In most case reports, medical management fails, resulting in debulking surgery (excisions, laser techniques, skin grafting) with mixed outcomes. ¹⁰

Unlike rhinophyma, conductive hearing loss from accumulation of desquamated debris or soft tissue stenosis of the EAC is a potential sequelae of otophyma. Pre and post-management audiograms can be used to measure outcomes and highlight the importance of comanagement between a dermatologist and an otolaryngologist. Specifically, examining the change in the air-bone gap as well as disimpaction frequencies can provide an objective verification of improvement in combination with the dermatologic and subjective improvements.

The comanagement approach relies upon canaloplasty to promote a self-cleaning ear followed by retinoid therapy, which acts 2-fold: to diminish the size and activity of sebaceous glands and to downregulate the inflammatory response by interfering with leukocyte chemotaxis. ^1,13 After this combined approach, our first patient had near complete closure of the air-bone gap bilaterally, signifying a substantial recovery of conductive hearing loss, improving by 49 dB in his right ear and 25 dB in his left ear. In contrast, the second patient had underlying middle ear disease with mixed hearing loss, distinct from his otophyma presentation. This complicated his posttreatment audiogram interpretation. Nonetheless, this case had a successful outcome indicated by a dry and patent ear canal and the patient’s ability to wear hearing aids without further inflammation or infection. The third patient did not have pre and post-management audiograms to compare; however, given the extensive frequency and large amount of debris removed during his disimpactions, it can be presumed he had some degree of conductive hearing loss. The patient did however have a marked improvement in his disimpaction frequencies. In the 12 months prior to comanagement, the patient had 8 bilateral disimpactions. In the 23 months post-management, he has required only 2 disimpactions and reports hearing improvement. All 3 patients were pleased with the resultant functional and physical appearance following this comanagement approach.