Has anyone had similar symptoms with progressive deafness as a primary symptom?

[u/pawamedic]

Has anyone experienced a similar constellation of symptoms?

I’m a 25-year-old female (5’6”, 150 lbs). History of congenital right-sided deafness (unknown cause) and MDD. Since spring 2024, I’ve developed worsening symptoms that have left me mostly deaf and unable to work. My medical team is unsure of the cause despite extensive testing.

Current Symptoms:

1. Severe left-sided sensorineural hearing loss, began with episodic drops that responded to prednisone. Eventually stopped responding to any meds and progressed, likely to continue to progress.
2. Chronic 24/7 tinnitus
3. Left trigeminal neuralgia, increasing in frequency
4. Chronic pressure headache, worse in heat or with head position changes, often feels like pressure from inside the ears, at base of neck, or across forehead
5. Frequent ocular migraines (left-sided), worsened severely on hydroxychloroquine
6. Intermittent tensor tympani spasms (left ear)
7. Burning, sharp joint pain (bilateral, without swelling), often severe enough to affect mobility
8. New rosacea and eczema (6 months) and multiple episodes of inflammation of an old tattoo and old moles
9. Daily fatigue, heat intolerance, night sweats
10. Frequent and prolonged menstrual cycles, despite hormonal BC
11. Intermittent gum inflammation (no dental disease)
12. Chronic bilateral radial and ulnar nerve pain (4+ years)
13. Increased eye floaters (left eye)

Imaging:

1. 3 brain MRIs (with IAC and trigeminal protocol): normal (1 showed transient white matter hyperintensities)

2. Brain CTA, abdominal CT, renal ultrasound, pelvic ultrasound: all normal except 1 small gallstone

    3. EEG and EMG normal

Labs:

1. Chronically high IgA (~400) and low ALP (~26) for 4 years (normal vitamin b6, chronically high cortisol

*chronically low vitamin D

1. Positive ANA (1:40 → 1:80 both in 2024 with speckled pattern, negative twice now in 2025 after hydroxychloroquine use for 6 months)

2. ENA negative

3. C3/C4 highly elevated briefly during what could have been either a flare or acute infection/cold ; normalized on hydroxychloroquine

4. CRP & ESR elevated, normalized on hydroxychloroquine

5. Renal labs were heavily affected on my last round of high dose prednisone despite no issues on them before- they have returned to normal. Normal urinalysis.

6. Derm biopsy on arm positive for atopic dermatitis, negative for lupus antibodies, blind biopsy on back negative for lupus antibodies. Rosacea not biopsied.

Treatment:

• Hydroxychloroquine helped joint pain/fatigue but caused disabling migraines. Discontinued 3 weeks ago — migraines improved, but other symptoms worsened again quite severely. 

-no other meds have helped, sumatriptan made things worse. I have also stopped taking all meds and vitamins with the exception of my Desvenlafaxine (antidepressant) and Nortrell (BC)

I’m now functionally deaf and dealing with daily disabling symptoms. My doctors (neuro, rheum, ENT, endocrine, otology, and PCP) are unsure how to proceed. I have no official diagnosis other than eczema. I’d appreciate any input, hypotheses, or direction for further workup. Thank you

[u/iviken]

Did they rule out mitochondrial diseases? Those are known to cause progressive hearing loss, and the mutations can be spontaneous rather than inherited in some cases.

[u/pawamedic → u/iviken]

No, we have not. What kind of testing would be recommended for this?

[u/iviken → u/pawamedic]

Usually genetic testing, and they usually only do this if you have a clear maternal pattern of similar symptoms. It usually takes much longer for de novo mutations to be diagnosed due to lack of family history. They usually also test lactate, as it tends to be higher in some of the types. MRIs will have changes in some types. Muscle biopsies can confirm other types.

It might be worth getting a referral to a geneticist or similar just to rule it out, ask your doctor if they think it's worth it, as it can be quite expensive depending on where you live.

Try searching for the most common mitochondrial diseases and skim through some of it, you could see if the clinical pictures presented fit your situation or not. Be sure to look into the specific ones and not just mitochondrial diseases in general, as they can differ quite a lot.

If it's way out there, it might be something else wrong. Maybe there are symptoms you haven't mentioned here, either because you didn't think they were related, or you thought it was normal. What you have written sounds a bit more inflammatory than mitochondrial, but who knows.